Our patient was a 68 - year - old woman with chronic obstructive pulmonary disease . She had no history of gastroesophageal reflux disease or esophageal disorders but was found to have a progressive solitary pulmonary nodule ( 1×1.3 cm ) . A right lower wedge resection was performed in December 2011 and the University of Iowa pathology department identified the tumor as a LCNEC . Approximately six months after her operation , our patient started having slowly progressing dysphagia for both solids and liquids . A positron emission tomography ( PET ) scan performed in 2012 , one year post surgery , was unremarkable for recurrence or metastasis . Repeat computed tomography ( CT ) in June 2013 ( 18 months post surgery ) did not show any recurrence ( Figure 1 ) . Our patient had an esophagogastroduodenoscopy ( EGD ) and barium swallow that revealed no stricture of her esophagus but failure of primary and secondary peristaltic waves and reflux . In July 2013 , esophageal manometry found failure of the lower esophageal sphincter to relax , with a residual pressure of 32mmHg . Our patient was treated with calcium channel blockers with partial relief . Thus far , up to 18 months post resection , our patient had been able to maintain her weight and her basic laboratory parameters , including hemoglobin , albumin and cholesterol levels . However , in January 2014 , she reported worsening dysphagia and weight loss of 10lbs over a period of one month . She was referred to our center for further treatment of her achalasia . A repeat esophageal manometry showed an elevated lower esophageal sphincter pressure of 50mmHg as well non - propagative , non - peristaltic contractions throughout her esophageal musculature ( Figure 2 ) . A repeat EGD with an intention to perform therapeutic pneumatic dilation showed a nearly circumferential ulcerated , fungating mass from approximately 36 cm that extended to her gastroesophageal junction at 39 cm . Biopsies revealed a LCNEC with similar features to the primary tumor resected in 2011 , and was concluded to be a metastasis . Subsequently , a repeat CT scan revealed progressive diffuse metastatic disease . Our patient underwent two courses of chemotherapy but died from the disease after six months .


We present a case of pancreatic tumor without a history of trauma or panceratitis . A 47 - year - old Tunisian man with a history of Crohn 's disease was admitted to the University Hospital in 2015 because of fluid chronic diarrhea with 4 stools per day daytime only with out ooddebr is associated with vomiting with out abdominal pain or fever with a weight loss not encrypted dating from 6 months . Laboratory tests were normal . Nonspecific elevations of serum pancreatic enzymes . Patient underwent an abdominal ultrasound and computed tomography ( CT ) that revealed : Aspect of ileitis of the last ileal loop extended by 300 mm with distended ileal loops upstream , cystic image at the tail of the pancreas with clean wall uncalcified and hypodense content of fluid density without endoluminal bourgeon or pancreatic duct dilatation , measuring 28 x 22 mm ( Figure 1 ) . The size of the head was normal . There were no enlarged lymph nodes ( Figure 2 ) . Aspect of bilateral sacroiliitis and bilateral coxarthrosis . MRI show an aspect of ileitis of the last ileal loop and the cystic nature of a pancreatic lesion , with parietal enhancement at the caudal portion of the pancreas with a slight dilation of the Wirsung . Otherwise , gallbladder contains multiple gallstones and biliary sludge . Tumor markers are normal.on the other hand , in colonoscopy terminal ileum appears swollen , inflamed , with frequent ulcerations and biopsies were made . The biopsies were taken and show a subacute and ulcerative ileitis compatible with Crohn 's disease . Esophagogastroduodenoscopy was normal . The patient was operated by laparoscopy , he had a caudal pancreatectomy and cholecystectomy .1.5 cm cystic tumor in the tail of pancreas was resected and sent for pathological examination . The post - operative course was uneventful . Patient was discharged home within 7 days in a good general condition . There were no symptoms of glucose intolerance after normal diet administration . The microscopic examination revealed well differentiated cystic neuro endocrine tumor of the tail of the pancreas grade I according to the classification of WHO 2010 ( mitosis < 2/10CFG and Ki67<2 % ) associated with neuroendocrine hyperplasia lesions of islets of Langerhans , surgical limit was healthy , pTNM pT2 , ectopic spleen , chronic cholecystitis . the patient come for out - patient consultations at the hospital , he felt well , with no severe complaints and with correct periodic findings and normal glucose metabolism .


A 41 - year - old Caucasian woman underwent a primary double lung transplantation for cystic fibrosis in 2006 . Her medical history was otherwise unremarkable and the patient had no known history of pulmonary tuberculosis or tuberculosis contact . On December 2015 , she underwent retransplantation for chronic lung allograft dysfunction . During the month preceding retransplantation , 4 sputum specimens remained negative for acid - fast bacilli and specific M. tuberculosis culture and real - time polymerase chain reaction ( PCR ) testing . On postoperative day 42 , deterioration of her respiratory status prompted a chest - computerized tomography ( CT ) scan revealing sub - centimeter bilateral nodules primarily located in the apical posterior lobes and a bilateral pleural effusion ( Fig.1 ) . The same day , a bronchoalveolar lavage ( BAL ) yielded a positive real - time PCR for rifampicin - susceptible M. tuberculosis , confirmed by culture on postoperative day 62 . Tuberculin skin test ( TST ) or interferon - γ release assay ( IGRA ) test were not performed . All the BALs performed on postoperative period yielded no other pathogen except for the one performed on day 60 that cultured Pseudomonas aeruginosa ; the adjunctive antibiotic therapy was imipenem - cilastatin , 3 g / d. Histological examination of a lung biopsy performed 6 weeks after retransplantation revealed a caseating granuloma and necrosis . Acid - fast bacilli were identified as rifampicin - susceptible M. tuberculosis by real - time PCR . On postoperative day 65 , the patient 's status worsened with severe hypoxemia , shock unresponsive to high dose cathecolamines , and multiorgan failure . The patient died on postoperative day 70 , despite treatment combining isoniazid , rifampicin , ethambutol , and pyrazinamide . Retrospective real - time PCR testing of the explanted lung and BALs performed on postoperative days 1 , 7 , and 21 remained negative . The organ donor died of posttraumatic intracerebral hemorrhage . He was a 47 - year - old man with no history of lung disease or risk factors for tuberculosis other than chronic alcohol use and smoking . TST results were not available . During hospitalization , a lung CT - scan showed no signs of active or previous tuberculosis and no TST or IGRA test results were available . Routine cultures of per - transplantation right lung biopsy yielded Candida albicans . Retrospective M. tuberculosis real - time PCR yielded negative results on the left and right donor - lung biopsies . Both kidneys from the same donor were transplanted into 2 other recipients . Six months after transplantation , neither of the kidney recipients had developed any signs or symptoms suggestive of active tuberculosis .


A 54 year - old diabetic woman complained of blurred vision in the left eye for 5 days without scalp tenderness or headache . Visual acuity ( VA ) was 20/20 right eye and 20/30 left eye . Intraocular pressures were normal . Funduscopic examination revealed left optic nerve edema and a small disc margin hemorrhage . WBC count was 12,700 , ESR was 7 ( normal 0–20 mm / hr ) and CRP was 2.4 ( normal 0–1.0 mg / dL ) . Three days later , she noted new left eye pain and her vision declined to counting fingers . There was no pain on eye movement . The right pupil was 4 mm and reacted to light ; the left pupil was 4 mm , and there was a relative left afferent pupillary defect . The left optic nerve was swollen with a few peripapillary nerve fiber layer hemorrhages along with a cherry red spot and mild macular edema consistent with central retinal artery occlusion ; there were no posterior pole or peripheral retinal hemorrhages . Trace cells were seen in the anterior chamber , but not in the vitreous fluid . Repeat WBC , ESR and CRP were normal . Treponemal and Bartonella antibodies , the QuantiFERON - TB test , blood cultures , EKG and carotid Doppler ultrasound were unremarkable . Brain contrast MRI demonstrated no optic nerve enhancement . Twelve days later , left eye pain and vision worsened , and the patient complained of jaw claudication and scalp pain . VA declined to no light perception in the left eye with 1 + cells and flare in anterior chamber and 2 + vitreous cells and haze . Funduscopic examination revealed extensive retinal necrosis and diffuse hemorrhages with multiple areas of focal venous beading ( Fig.1 ) , consistent with acute retinal necrosis . Vitreous fluid was examined by PCR for amplifiable HSV , VZV , CMV and toxoplasma sequences and returned positive for VZV DNA . She was treated with a one - time intravitreal injection of ganciclovir 2000 mcg/0.5 ml in the left eye , oral acyclovir 800 mg 5 times daily for 14 days and oral prednisone 60 mg daily for 7 days followed by 20 mg for 7 days . Eleven days after starting treatment , because she had recently developed jaw claudication and intermittent scalp pain , a temporal artery biopsy was performed . Histological examination was normal ( Fig.2 ) while immunohistochemical analysis demonstrated VZV antigen in the arterial adventitia ; evaluation of adjacent sections for HSV-1 antigen and infiltrating leukocytes ( CD45 , not shown ) was negative ( Fig.3 ) . Antiviral therapy was altered to intravenous acyclovir , 10 mg / kg every 8 h for 14 days , and steroids were discontinued .


This is a 53 - year - old male patient who went to our hospital with chief complaint of massive gum bleeding for 1 day . The patient had a history of chronic hepatitis C. PEG - IFN - α-2a ( 180 μg ) plus ribavirin ( 1200 mg / day ) were prescribed to the patient since March 17 , 2014 . He denied any autoimmune conditions before treatment . The baseline virological data revealed high virus load ( HCV RNA 2.1 × 106 IU / mL ) with genotype 1b . Rapid virological response was not achieved at the fourth week ( HCV RNA : 2.12 × 106 IU / mL ) . Partial early virological response ( HCV RNA : 103 IU / mL at week 12 ) and delayed virological response ( HCV RNA : < 15 IU / mL at Week 24 ) were noted . However , fatigue , anemia , and depression syndrome were progressed at 30th week . The patient requested to stop treatment at Week 36 . The viral load at the end - of - treatment ( EOT ) was undetectable . The platelet count at EOT was 92 × 103 cells / μL and elevated to 159 × 103 cells / μL 1 week later . Two weeks following EOT , the patient developed massive gum bleeding . The physical examination showed the multiple petechiae on the extremities . There is no sign of intracerebral hemorrhage , gastrointestinal bleeding , or other internal bleeding . The initial platelet count was 4 × 103cells / μL. Coagulation profile showed normal prothrombin time , activated partial thromboplastin time , fibrinogen , d - dimer , and fibrin degradation product . Peripheral blood smear showed neither fragmented red blood cells , helmet cells nor abnormal platelet aggregation . Concomitant autoimmune connective tissue diseases such as systemic lupus erythematosus or cryoglobulinemia were excluded due to negative anti - nuclear antibody and cryoglobulin except for positive anti - cardiolipin IgG ( 116 GPL , normal range < 20 GPL ) and anti - phospholipid IgG ( 165 U , normal range < 15 U ) . Anti - phospholipid syndrome was excluded due to no previous thromboembolic events , according to 2006 Sapporo criteria.12 Bone marrow biopsy was also performed , which revealed hypocellular marrow with even cellular distribution and without evidence of lymphoid neoplasia . The potential drugs that may cause platelet lysis were ruled out . Blood transfusion of platelet was performed , but poor response with rapid decline of platelet count in the next day of transfusion . A diagnosis of immune thrombocytopenic purpura was made . We started intravenous methylprednisolone therapy ( 40 mg , 3 times daily ) on November 27 combined with platelet transfusion treatments . It still showed no significant improvement . Azathioprine ( 100 mg , oral , once daily ) was added since December 3 . Hydroxychloroquine ( 400 mg , oral , once daily ) was also prescribed due to positive anti - phospholipid antibodies . Platelet count gradually increased to 93 × 103 cells / μL 17 days after admission , so methylprednisolone was changed to oral form and slowly tapered off . On December 15 , the patient was discharged due to stable condition with platelet counts elevating to 117 × 103 cells / μL ( Figure 1 ) . Followed laboratory data after 5 months revealed sustained virologic response , platelet count above 150 × 103 cells / μL , and decreased anti - cardiolipin IgG ( 30.5GPL ) and anti - phospholipid IgG ( 73.21 U ) levels .


A 65 - year old female was admitted with progressive dysphagia for 2 months and 5 kg weight loss . Her past medical history was significant for osteoporosis treated with calcium lactate tablets , daily , for 5 years . Upper gastrointestinal endoscopy described a 4 cm whitish firm mass in the middle esophagus ( Fig.1 ) and a semi - circumferential deep ulcer with irregular borders on the opposite mucosa ( Fig.2 ) . During endoscopy a pedicle was not identify by handling a polipectomy snare around the esophageal mass . Upper gastrointestinal series with gastrografin ( Fig.3 ) revealed an ovoid lacunar image at the distal part of the esophagus esophageal , inhomogeneous , with calcifications and smooth contours . During peristalsis the image was mobile and no pedicle was identified . The esophageal lumen was enlarged with a diverticula development at the posterior wall . Also computer tomography of the thorax excluded a pedunculated tumor , describing an intra - luminal calcified esophageal mass ( Fig.4 ) . The biopsies obtained from the esophageal ulcerated mucosa revealed inflammatory cells , without malignancy . Based on these endoscopic and imagistic results a bezoar was supposed to have been developed in an esophageal diverticula , subsequently with ulcerated mucosa . The esophageal mass was removed with an endoscopic snare in one piece , as the fragmentation was not physically possible . The macroscopic appearance revealed a 4 cm , globular mass , heterogeneous , dense , whitish , in places with harsh yellow foci , most likely dystrophic calcification . The macroscopic examination on cross section revealed a light gray aspect ( fibrous appearance ) that includes multiple harsh yellow - orange structures , difficult to section ( Fig.5 ) . This mass was immersed into trichloracetic acid for decalcification . Microscopic examination revealed hyaline fibrous tissue ( Fig.6a ) , stained in green in Tricrom Mason ( Fig.6b ) , with numerous crystalline basophils deposits of minerals , rare fibrocytes and very few vessels . The presence of capillary structures , rare fibroblasts and collagen fibers brought in discussion a mesenchymal originating mass , most likely a fibrovascular polyp . A definitive histological diagnosis was not possible , as the pedicle was not identified , but the presence of the connective tissue suggested the previous presence of a pedicle into the lesion . The long term calcium tablets intake might explain the calcification process developed into the vascular - connective tissue , revealed on histology by the numerous crystalline basophils deposits of minerals . One month later the patient was asymptomatic . The endoscopy did not revealed an enlarged esophagus , the appearance of the esophageal mucosa was normal ( without ulcerations ) and no diverticula was identified . No motility disturbances were found on esophageal manometry . Based on the clinical course , the history of calcium lactate intake and histological appearance a diagnosis of an esophageal benign mesenchymal originating mass ( most probably a fibrovascular polyp ) auto - amputated and calcified was formulated .


A 58 - year - old cotton farmer was presented to the West China Hospital of Sichuan University because of an over 1 - month history of recurrent fever ( between 38 and 40 ° C ) , productive cough , and dyspnea . Prior to admission , he was diagnosed of pneumonia and treated with latamoxef , ofloxacin , vancomycin , and voriconazole at local hospital . However , no remission of symptoms was observed . Moreover , he was a hepatitis B virus carrier with a 10 pack - years smoking history . However , no history of diabetes mellitus , tuberculosis , and use of glucocorticoids in the past were informed . On admission , blood pressure , 145/95 mm Hg ; heart rate , 120 per minute ; respiratory rate , 30 per minute ; and temperature , 39.3 ° C . Physical examination revealed diminished breath sounds , but no rales were heard , and evaluation of other systems was unremarkable except moderate edema of lower limbs . Arterial blood gases analysis showed pH 7.361 , PCO2 53.5 mm Hg , and PO2 62.8 mm Hg . Laboratory data ( Table (Table1)1 ) revealed leukocytosis of 49,500 / mm3 with 97.4 % neutrophils , and elevated procalcitonin of 5.16 ng / mL. Chest computed tomography , presence of nodules , masses , patchy consolidations , and bilateral pleural effusion , is noted ( Fig.1A ) . Meanwhile , it was soon alerted in sputum smear with presence of filamentous , gram - positive , weakly acid - fast , and beaded bacilli with possible diagnosis of Nocardia infection ( Fig.1B , C ) . Trimethoprim - sulfamethoxazole ( 3 pills per 6 hours ) with noninvasive ventilation was promptly administered . Sputum culture showed growth of numerous bacteria that were precisely determined to be N otitidiscaviarum by the method of mass spectroscopy on day 6 after admission ( Fig.1D ) . Antibiotics were thus modified to amikacin and imipenem in addition to trimethoprim - sulfamethoxazole in accordance with the sensitivity test . However , the patient was not improved as expected and eventually died from severe respiratory insufficiency on the 13th hospital day .


A 18 - year - old man presented with a history of a palpable lump in the presternal area , which was found incidentally after weight reduction . The patient had no other relevant medical or trauma history . On physical examination , the lesion was found to be a nonmovable , firm mass with no tenderness or associated skin changes , detected at the midline position over the sternum ( at the manubrium level ) . There was no visible fistulous opening or discharge from the lesion . On ultrasonography , we detected a well - circumscribed , oval , anechoic mass , with posterior acoustic enhancement , that measured about 3.3 × 1.7 × 3.1 cm , and was located in the subcutaneous fat layer over the sternum . In the dependent portion of the mass was an internal , well - circumscribed , heterogeneously hypoechoic , egg - shaped lesion ( Fig.1A and B ) showing a movement according to patient movement . The mass could be compressed using the linear transducer ( Fig.2A and B ) . A color Doppler study showed no vascularity within the cystic mass or the internal hypoechoic lesion ( Fig.1C ) . Surgical excision of the mass was performed without postoperative complications . Grossly , the excised mass was a well - defined , ovoid , cystic mass gray - tan in color . On section , it was found to be a unilocular cyst filled with whitish mucous material . Microscopically , the mass was lined with ciliated pseudostratified columnar epithelium suggestive of respiratory type - mucosa ( Fig.3 ) . There was nonspecific collagenous fibrosis around the cyst . The differential diagnosis of subcutaneous cyst included epidermal inclusion cyst , thyroglossal duct cyst , branchial cleft cyst , and dermoid cyst . There was no histological evidence of squamous epithelium , keratin , thyroid tissue , or skin appendage in the cystic wall of the present case . The mass was diagnosed as a bronchogenic cyst .


A 23 - year - old woman with a history of carnitine deficiency presented with exertional dyspnea and fatigue . She was first diagnosed with nonischemic cardiomyopathy at age 10 years , when she presented with symptoms of congestive heart failure and transthoracic echocardiography ( TTE ) revealed severe left ventricular ( LV ) dysfunction . At that time , her total carnitine level was <3 μmol / L ( normal range , 25–69 μmol / L ) , and her free carnitine level was <3 μmol / L ( normal range , 16–60 μmol / L ) . The cardiomyopathy was attributed to carnitine deficiency . After 5 months of therapy with carnitine supplements , her total carnitine and free carnitine levels had increased to 86 μmol / L and 48 μmol / L , respectively , and her left ventricular systolic function had normalized , with fractional shortening of 34.7 % . At the current admission , the patient reported that , over the past 10 years , she had only been intermittently compliant with her carnitine supplement regimen , and that , approximately 6 months before presentation , her total carnitine and free carnitine levels had dropped to 18 μmol / L and 17 μmol / L , respectively . She underwent TTE followed by cardiac magnetic resonance ( CMR ) with gadolinium enhancement . The TTE showed normal LV function and mild , concentric LV hypertrophy ( Fig.1 ) . Results of CMR confirmed normal LV size and function but showed focal increased wall thickness at the basal and mid lateral wall ( end - diastolic thickness , 1.6 cm ) ( Fig.2 ) with patchy delayed gadolinium enhancement ( Fig.3 ) . On the basis of the patient 's family history of sudden cardiac death , abnormal myocardial structure , and underlying disease process , a dual - chamber implantable cardioverter - defibrillator was placed for primary prevention of sudden cardiac death from both bradyarrhythmias and tachyarrhythmias .


A 50 - year - old lady presented with complaints of pain abdomen and jaundice for one and a half months ; she also complained of intermittent type of fever in the last 1 week . Physical examination revealed presence of icterus and a palpable tender globular mass in the right hypochondrium measuring 3 × 3 cm . A clinical diagnosis of obstructive jaundice secondary to periampullary carcinoma was made . Ultrasonographic examination of the abdomen and pelvis revealed dilatation of the intrahepatic biliary radicles ( IHBR ) , with multiple freely mobile gall stones and a dilated common bile duct ( CBD ) measuring 1.8 cm with sudden narrowing at its distal end . CT scan showed moderate dilatation of the extra and intrahepatic biliary radicles , cholelithiasis with suspicious thickening of the gall bladder wall at the fundus , and a distal CBD stricture . There was no evidence of any distant metastasis ( Fig.1 ) . A preoperative diagnosis of periampullary carcinoma with chronic cholecystitis was made , and the patient planned for a pancreaticoduodenectomy ( Whipple ’s procedure ) after optimization . The patient underwent a classical Whipple ’s procedure ; table examination of the resected specimen revealed multiple gall stones in the gall bladder with area of mucosal thickening at the fundus and an irregular circumferential growth involving the intrapancreatic portion of the CBD . Postoperative period was uneventful , and the patient recovered satisfactorily . Histopathological examination of the resected specimen ( Figs.2 and 3 ) revealed thickening of the gall bladder wall at the fundus with multiple small calculi , the largest measuring 1.4 cm ( Fig.3 ) . Sections studied from the area of thickening showed features suggestive of adenocarcinoma limited to the mucosa . Microscopic sections obtained from the distal CBD region showed adenocarcinoma of the common bile duct with invasion into the muscular wall of the duodenum . Sections studied from the resected lymph nodes were free of tumor cells . A pathological staging of pT1aN0M0 for adenocarcinoma of the gall bladder and pT2N0M0 for adenocarcinoma of the distal CBD was made , and the patient was not subjected to any adjuvant therapy . The patient was disease - free 2 years after surgery .


A 68 - year - old female smoker with a history of pulmonary embolism and diabetes mellitus was diagnosed with Stage IIIB ( T4N2M0 ) squamous NSCLC . She was treated by definitive chemoradiotherapy with cisplatin and vinorelbine until September 2014 . In October 2014 , positron emission tomography – computed tomography ( PET - CT ) scan demonstrated a good response in the primary lesion ; however , new metastases in the right adrenal gland and right femur developed and were irradiated in November 2014 . A month later the disease progressed with development of multiple bone and subcutaneous metastases . At that point , the patient suffered from severe dyspnea and was oxygen - dependent . She received one cycle of carboplatin and gemcitabine followed by severe pancytopenia , and treatment was switched to nivolumab 3 mg / kg q14 days in January 2015 . One week after the first cycle of nivolumab , a subcutaneous lesion in her upper back grew substantially , accompanied by severe pain and significant inflammatory reaction ( Fig.1 ) . Other subcutaneous metastases grew slightly as well . After the second cycle of treatment marked symptomatic improvement was observed , including improvement in general appearance and dyspnea and reduction of the bone pain . The patient no longer required oxygen supplementation . The subcutaneous lesions started to regress too , with complete resolution by the 12th week as well as improvement in all bone lesions ( Figs.1 and 2 ) . The patient continued on nivolumab until June 2015 , and tolerated the treatment well . Unfortunately , she developed bacterial aspiration pneumonia and passed away in June 2015 .


A 54 - year - old patient was admitted for fever , diarrhea , and acute renal failure . He had no significant medical history , did not take any medication , and had not traveled recently . He had no relevant familial history . Fifteen days before his admission , he developed fever , fluctuant rash , diarrhea , and pain in the joints . On admission , blood pressure was 140/70 mm Hg , temperature 39 ° C . Physical examination showed severe edema involving both lower and upper limbs , as well as ascites and pleural effusion . Cervical and axillar infracentimetric lymphadenopathies were present , together with hepatosplenomegaly , confirmed by computed tomography ( CT ) scan . Bilateral arthritis of the ankles resolved spontaneously . Blood analysis revealed ( Table (Table1):1 ): leukocytosis , normal hemoglobin and platelet levels with no biologic sign of hemolysis , elevated C - reactive protein , low albumin , elevated serum creatinin , mild proteinuria and no hematuria . Despite intravenous rehydration using saline and albumin , renal function worsened and the patient required dialysis . While diagnostic investigations were performed , hemiplegia occurred : brain MRI showed multifocal ischemic lesions . Echographic and rythmologic studies ruled out any cardiologic cause for the stroke . A renal biopsy was performed ( Fig.1A and B ) . Periodic acid – Schiff ( PAS ) staining showed endotheliosis in all glomeruli ( Fig.1A , arrows ) , associated with mesangiolysis and double contours on silver staining ( Fig.1B , arrows ) , and no arteriolar thrombus . A moderate CD20 + B lymphocyte infiltrate was present in the interstitium , with a peritubularcapillaritis . Immunofluorescence study did not show any deposit . Lymph node biopsy ( Fig.2A and B ) showed abnormal follicles with hyalinization of germinal center , and an onion - skin aspect of the mantle zone . Clinical presentation and lymph node histology were consistent with hyaline - vascular multicentric Castleman disease ( MCD ) . HIV and HHV-8 serology , as well as HHV-8 lymph node tissue staining , were negative . Serum vascular endothelium growth factor ( VEGF ) was highly elevated . There was no biologic manifestation of thrombotic microangiopathy ( TMA ) , but the renal biopsy and brain MRI were consistent with glomerular and neurologic TMA lesions . No other apparent cause of thrombotic microvascular involvement was noted . Notably , there was no evidence for infection with shiga toxin - producing germs , ADAMTS-13 activity was decreased up to 14 % but remained above 5 % , and the alternative complement pathway was normal . Plasma exchange was initiated due to the kidney histological lesions and the multifocal ischemic brain lesions . It was discontinued after 1 month after the onset of the disease , since the patient 's condition remained stable . Furthermore , chemotherapy including 6 courses of rituximab ( 375 mg / m2 ) , cyclophosphamide ( 750 mg / m2 ) , and dexamethasone ( 40 mg / day from day 1 to day 4 ) was started together with plasma exchanges ( chemotherapy was performed immediately after plasma exchanges ) . Courses were performed every 3 weeks . Clinical manifestations of vascular leak syndrome regressed , renal function normalized , and serum VEGF level decreased to 825 pg / mL after 1 course of chemotherapy . The patient was discharged 43 days after admission . No neurological event occurred after initiating the treatment . Thoraco - abdominal CT scan was performed after 6 courses of chemotherapy , showing a normal liver and spleen size and no lymph node enlargement . One year after the diagnosis , remission of MCD is persistent and plasma creatinine is 86 μmol / L , with no proteinuria .


A 60 - year - old woman patient was admitted to our hospital on Feb. 18 , 2016 because of frequent episodes of hemoptysis for 2 weeks . In the previous decade , the patient had frequent but less severe episodes of hemoptysis , which typically ensued following an upper respiratory tract infection . Ear , nose , and throat examination and laryngoscope failed to identify an apparent source of bleeding . CT chest scan revealed no abnormality . The most recent episode occurred after an upper respiratory tract infection 2 weeks ago . The patient coughed up as much as about 500 mL of fresh blood in an episode of hemoptysis , which was alleviated by anti - tussive therapy . The patient denied a history of smoking and illicit drug use . Diagnostic workup revealed no evidence of coagulopathy . Upon admission , physical examination revealed signs of rhonchi and reduced breath sounds . Bronchoscopy showed a 1 - cm lesion at the membranous trachea 2 cm to the carina . Tortuous blood vessels were observed running in the submucosa of the trachea ( Fig.1 ) . CT angiography was performed , demonstrating an artery extending into the submucosa from the descending aorta ( Fig.2 ) . A diagnosis of Dieulafoy disease of the trachea was entertained . Since the blood vessel was considered to be the culprit of hemoptysis , selective arterial embolization was performed 1 week later . No fresh episode of acute hemoptysis was observed and the patient was still being followed up at the time of writing this report . This study was approved by Ethics Committee of Shanghai Jiao Tong University Affiliated Sixth People 's Hospital , and also got an informed written consent from the patient .


A female outpatient in her 50s had routinely visited our hospital because of liver cirrhosis resulting from hepatitis B virus and biliary duct stones . At 65 years of age , she suffered from HCC with BCLC early stage A. Her liver function was well preserved , and Child - Pugh score was A. She underwent left lateral segmentectomy for HCC , without other preoperative treatments . Histopathological findings revealed bridging fibrosis and pseudolobule formation . Serum levels of alpha - fetoprotein ( AFP ) and protein induced by vitamin K absence - II normalized after primary resection ( Figure 1 ) . In imaging studies , no LN metastasis was detected at the time of primary resection of HCC . Two and a half years postoperatively , the patient ’s AFP level increased dramatically to 780.2 ng / ml . A lobular lesion with fine enhancement was detected by contrast - enhanced magnetic resonance imaging . The tumor measured 27 mm in diameter and was located caudally on the left kidney . In contrast - enhanced computed tomography , the tumor showed strong enhancement in the arterial phase ( Figure 2A , 2B ) and a relatively low density in the portal phase ( Figure 2C , 2D ) . These enhancement findings appeared consistent with a typical HCC pattern . Detailed imaging studies of both magnetic resonance imaging and computed tomography were performed in this case because a very rare metastatic LN initially seemed to be debatable and we needed to rule out exclusion diagnoses . Three - dimensional imaging proved that the tumor was fed by a main vessel from the inferior mesenteric artery ( red arrow ) and by an accessory feeder from the superior mesenteric artery ( Figure 3 ) . Fluorine-18 - fluorodeoxyglucose positron emission tomography ( FDG - PET ) and positron emission tomography - computed tomography did not detect the tumor ( Figure 4 ) , although we thought positron emission tomography - computed tomography was helpful to identify other metastatic tumors . Further imaging findings revealed no other intrahepatic or extrahepatic metastasis . Based on the tumor location , the clinical diagnosis was solitary metastasis to a mesocolic LN or HCC dissemination . Determining the ideal therapeutic strategy for solitary but extrahepatic rare metastasis was difficult . Although rapid growth was a critical concern in this case , the tumor was solitary and not accompanied by other metastases . Considering both diagnostic and therapeutic viewpoints , we finally chose surgical resection in this case . No disseminative nodules , lymphadenopathy , or ascites was observed during surgery . The tumor was located in the mesocolon nearly at the wall of the descending colon , and partial resection of the descending colon with regional mesocolon was performed . The patient ’s postoperative course was uneventful , and she was discharged on postoperative day 8 . Serum levels of tumor marker decreased immediately after surgery ( Figure 1 ) . Macroscopically , the mesocolic tumor was a solid and elastic mass with a smooth surface ( Figure 5A ) . A yellowish nodule was encapsulated in the cut surface ( Figure 5B ) . The enlarged LN contained metastatic HCC with a ductal structure ( Figure 6A ) , and immunohistochemically , the tumor was positive for AFP and negative for CK-20 , which was consistent with the pattern of primary HCC ( Figure 6B ) . The histopathological diagnosis was metastatic HCC to a mesocolic LN . As of the writing of this report , the patient has remained free of recurrence for 13 months after the second surgery , and has also been carefully followed up . No adjuvant therapies have been performed .


A 63 - year - old woman with no known cardiac history presented with a sudden onset of dyspnea requiring intubation and ventilatory support out of hospital . She denied preceding symptoms of chest discomfort , palpitations , syncope or infection . The patient was afebrile and normotensive , with a sinus tachycardia of 140 beats / min . The cardiorespiratory examination was remarkable for an elevated jugular venous pressure at the angle of the jaw , a left - sided third heart sound and bibasilar crackles in both lung fields . The complete blood cell count , electrolytes and cardiac biomarkers were within normal limits . An initial electrocardiogram revealed ST depression in leads V2 to V6 , suggestive of anterolateral ischemia ( Figure 1 ) . A chest radiograph demonstrated pulmonary vascular congestion consistent with a diagnosis of pulmonary edema ( Figure 2 ) . An echocardiographic examination revealed normal cardiac dimensions , normal wall motion and mild diastolic dysfunction with an early diastolic to late diastolic transmitral ratio of less than 1 , prolonged deceleration time ( 250 ms ) and a reduced early diastolic annular velocity of the lateral mitral valve annulus . Following aggressive diuresis and requiring minimal ventilatory support , the patient was extubated one day following her initial presentation . Within 1 h of extubation , the patient developed recurrent pulmonary edema clinically and radiographically , requiring mechanical ventilation . Cardiac catheterization was performed and revealed normal coronary arteries . On day 4 of the coronary care unit admission , the patient failed a repeat attempt at extubation . A computed tomographic scan revealed a 4 cm × 9 cm multinodular goiter extending into the mediastinum and suspected associated tracheal stenosis ( Figure 3 ) . With a diagnosis of NPPE secondary to an enlarged goiter , the patient underwent surgical intervention for a thyroidectomy . Intraoperative bronchoscopy revealed tracheal stenosis of greater than 50 % . The postoperative course was uncomplicated , and three months following discharge , the patient returned to her previous activity level without subjective dyspnea .


Our patient was a 50 - year old man with bicuspid aortic valve insufficiency and ascending aorta dilatation . He had undergone a free - hand aortic homograft implantation with replacement of the non - coronary sinus using a 24 - mm homograft in 2006 ( at the age of 41 ) . Follow - up evaluations were performed every 6 months and showed mild aortic valve insufficiency . After 8 years of follow - up , the patient presented to hospital with fever and the echocardiography showed severe aortic insufficiency due to a rupture of one of the cusps of the homograft , worsening of cardiac contractility with left ventricular ejection fraction ( LVEF ) depression ( 40–45 % ) and dilatation of the left ventricle ( telediastolic / telesystolic diameters 60/42 mm , respectively ) . The patient presented orthopnoea and hypotension , which needed infusion of dopamine and diuretics . Computed tomography scan showed calcifications on the homograft , pleural effusions and no signs of pericardial effusion ( Fig.1 ) . Since the Dukes criteria for endocarditis were not satisfied , the final diagnosis was an early structural deterioration of the homograft , due to a severe calcification process . The operation was performed through median resternotomy and with standard aorta - right atrium central cannulation for the extracorporeal circulation . The leaflets and the annulus of the homograft presented severe calcifications and no signs of endocarditis were found . An Edwards Intuity bioprosthesis ( 21 mm ) was then implanted ( Fig.2 ) . The cross - clamping time was 41 min , and the cardiopulmonary bypass time was 64 min . The patient required dopamine and adrenaline at low doses for bypass weaning . No complications occurred during the postoperative period . Echocardiography before the discharge showed the absence of paravalvular leaks , a peak / mean aortic gradient of 34/20 mmHg and an LVEF of 33 % . Follow - up echocardiography after 3 months from the operation showed neither leaks nor malfunctions of the Edwards Intuity prosthesis , a peak / mean gradient of 38/22 mmHg and initial recovery of the LVEF ( valued 47 % ) . Patient was asymptomatic again .


A 52 - year - old man ( body surface area : 1.3 m2 ) known to have dilated cardiomyopathy ( DCM ) , was admitted for worsening heart failure ( NYHA class IV ) . He presented with a 5 - month history of cough , progressive dyspnoea , orthopnea and recurrent upper abdominal pain started from 2 months ago . On examination his vital signs were body temperature 37 ° C , blood pressure 80/40 mmHg , respiratory rate 24 breaths per minute , and electrocardiogram showed atrial fibrillation with heart rate 50 beats per minute . Physical examination revealed the jugular venous distension , significant tender hepatomegaly and bilateral pitting edema at lower limbs . Laboratory tests showed elevated pro - NT brain natriuretic peptide of 22145 pg / ml ( normal 0 to 227 pg / ml ) and unremarkable D - dimer . X - ray and computed tomography of the chest demonstrated consolidation of bilateral lower lobes with pleural effusion , while his venous Doppler of lower extremities was normal . Based on his clinical condition , echocardiography was immediately inserted . There were severe dilated cardiac chambers , especially enlargement of the left ventricle ( LV ) ( 58 mm / m2 ) with spherical shape , decreased wall thickness , impaired ejection fraction 22 % and severe mitral regurgitation on two - dimensional transthoracic echocardiography ( 2DTTE ) . Parasternal short axis and subxyphoid view ( Figure 1 ) showed the mobile right atrial mass highly suspicious of a thrombus traversing the right atrial cavity during the cardiac cycle accompanying with free - floating small parts of the thrombi . Real - time three dimensional transthoracic echocardiography ( RT-3DTTE ) was performed to further confirm the nature of mass . It showed a highly mobile thrombus , irregular in contour , measured 2.6 × 1.0 cm which floating around the orifice of inferior vena cava and protruding into the right atrial cavity ( Figure 2 ) . In addition , RT-3DTTE evaluated right ventricle ( RV ) systolic dysfunction with ejection fraction 15.7 % ( Figure 3 ) . He was maintained on digoxin , spironolactone , furosemide , sotalol and dopamine . At the same time therapeutic anticoagulation was started with low - molecular - weight heparin and warfarin . The patient had an uneventful hospital course and one week follow - up echocardiography confirmed adequate removal of the thrombus ( Figures 4A and 4B ) .


A 63 - year - old male patient without smoking or drinking history was admitted on June 11 , 2014 . His major complaints were gradually aggravated dysphagia and fatigue , on suspicion of obstructive disease in upper digestive tract . He had been an athlete before , and then retired in good physical status before admission . His family and social history indicated nothing abnormal . Thorough physical examination of his skin , oral mucosa , eyes , and genital areas failed to identify any superficial lesions . Additionally , laboratory tests including hepatic function , renal function , and serum tumor markers such as carcinoembryonic antigen , cytokeratin 19 fragment , squamous cell carcinoma , neuron - specific enolase , and carbohydrate antigen 125 were all in normal range . Therefore , further endoscopic and radiological examinations were carried out for accurate diagnosis . Endoscopic examination revealed a slightly pigmented , irregular mass , which was located in lower esophagus , measuring 5.0 cm × 3.0 cm in size . Fine needle biopsy of the lesion revealed esophageal melanoma , which was confirmed by histopathology . Besides chest and abdomen computed tomography ( CT ) , enhanced cranial magnetic resonance image ( MRI ) and bone emission computed tomography ( ECT ) showed enlarged mediastinal , nd also celiac lymph nodes ( Fig.1A ) , without obvious involvement of supraclavicular lymph nodes . Concurrently , the CT showed an isolated , irregular pulmonary tumor ( Fig.1B ) . Positron emission tomography was not carried out , because it was not covered by health insurance of this patient . Therefore , this patient was clinically staged as cT3NxM1 according to the 7th edition of American Joint Committee on Cancer TNM staging system for esophageal cancer . CT - guided percutaneous pulmonary biopsy was avoided , with the aim to diminish the risk of tumor dissemination . Single - stage resection of the esophageal and pulmonary lesions was assumed to be reasonable after multidisciplinary consultation , which was approved by Ethical Committee of Xuzhou Central Hospital . Because the prognosis of this patient probably was extremely poor without targeted antibodies , which he could not afford for financial reasons . After his informed consent , simultaneous Ivor - Lewis esophagectomy and right upper lobectomy were performed successfully , under general anesthesia , after double - lumen endotracheal intubation , followed by systemic dissection of lymph nodes located in mediastinum and abdomen , in accordance with the principles of oncological surgery . The operation time was 290 minutes , without obvious bleeding during the surgery . Postoperative pathological staining of the specimen revealed pleomorphic cells and abundant melanin granules ( Fig.1C ) , whereas immunohistochemical tests demonstrated positive expression of human melanoma black 45 ( HMB45 ) , microtubule - associated protein tau 1 ( MAPT1 ) , melan A and S100 , and negative expression of desmin , synaptophysin , and epithelial membrane antigen ( EMA ) , which was consistent with melanoma . The resection margin and dissected lymph nodes were pathologically tumor - negative . In addition , molecular study of the patient indicated mutation of V - raf murine sarcoma viral oncogene homolog B1 ( BRAF ) V600E. Based on these findings , a diagnosis of advanced PMME was tentatively established as stage IV ( pT3N0M1 ) , because there was insufficient evidence to distinguish synchronous primary pulmonary melanoma from metastasis for this patient . The postoperative recovery was mainly uneventful , and the patient was discharged 14 days after surgery . Subsequently , 4 cycles of adjuvant conventional chemotherapy with an interval of 3 weeks were completed , with controlled moderate toxic effects including thrombocytopenia , leukopenia , nausea , vomiting , and diarrhea . The detailed chemotherapy regimen is as follows : paclitaxel liposome for injection on day 1 and day 8 ( 135 mg per square meter of body surface area ; Nanjing Luye Sike Pharmaceutical Co. , Ltd. , Jiangsu , China . ) , tegafur injection on day 2 to 4 ( 1000 mg per square meter of body - surface area ; Shandong Qilu Pharmaceutical Co. , Ltd. , Jinan , China . ) plus cis - platinum on day 2 to 3 ( 75 mg per square meter of body - surface area ; Shandong Qilu Pharmaceutical Co. , Ltd. , Jinan , China ) . This patient suffered from moderate leukopenia/ myelosuppression after the second cycle of TPF chemotherapy , and he recovered quickly after the administration of granulocyte colony - stimulating factor ( G - CSF ) . Concurrently , recombinant human interferon alpha-2b ( Harbin pharmaceutical group biological engineering Co. , Ltd , Harbin , China ) was administrated via hypodermic injection thereafter ( 6000 units every 3 days ; Fig.2 ) , lasting for 1 year . The patient was followed up continuously after the surgery . Chest and abdomen CT , cranial MRI , bone ECT , and thorough physical examination were carried out every 3 months . Encouragingly , the patient survived without loco - regional recurrence or remote metastasis during the follow - up of two and a half years up to now ( Fig.3 ) .


The patient , a 9 year - old Caucasian female , was first diagnosed with medulloblastoma at age 3 years . Treatment included surgical resection of the tumor followed by systemic chemotherapy ( Vincristine , Lomustine , Cisplatin , Carboplatin , Cyclophosphamide , Etoposide ) , and craniospinal radiation ( 2,340 cGy ) with boost to the posterior fossa ( total dose 5,900 cGy ) . Cis - RA was subsequently administered for eleven , 14 - day cycles ( 180mg / m2 per day ) , over a 13 - month period for a cumulative dose of 17,360 mg ( 24,640 mg / m2 ) . She completed medulloblastoma therapy at age 5.6 years . At age 6 years , she was diagnosed with radiation - induced central hypothyroidism and growth hormone deficiency ( GHD ) . She was treated with levothyroxine with subsequent biochemical euthyroidism . Given tumor remission status and an abnormal height velocity of 0.94 cm / year , growth hormone ( GH ) therapy was initiated at age 7 years ( 0.3 mg / kg / week ) with subsequent increased height velocity of 3.6 cm growth / seven months ; however , seven months after initiation of GH , increased bony prominence was noted at wrists and knees , concomitant with declining height velocity ( 1.6 cm growth / five months ) . Physical examination and radiographs were significant for leg length discrepancy : the right lower extremity measured 1.75 cm shorter than the left ( right femur 24.9 cm , right tibia 19.6 cm , right lower extremity 44.5 cm , left femur 25.25 cm , left tibia 21 cm , and left lower extremity 46.25 cm ) . Prior to initiation of GH , height ( 102 cm ) ( Figure 1 ) and armspan ( 102 cm ) were similar , with upper segment / lower segment ratio of one ; seven months after GH , height increased minimally ( 105.6 cm ) compared to the more significant increase in armspan ( 109.2 cm ) , with upper segment / lower segment ratio remaining at one . Radiographs revealed poorly visualized growth plates in both distal and proximal femurs and tibias ( Figure 2A - B ) , raising concern for premature closure of the physes . This lower limb growth plate closure would normally be observed at the time of late - stage puberty . By contrast , all bone ages using left hand and wrist radiographs over the preceding two years displayed open growth plates ( Figure 2C - D ) .


A 21 - year - old male presented with a 2 - year history of progressive shortness of breath on exertion and dry cough . At physical examination , auscultation of the lungs has revealed random wheezes and coarse crackles . Cardiac auscultation was normal , and no cyanosis or peripheral edema was observed . There was no history of smoking or previous known pulmonary disease . On routine blood examination , blood counts and serum chemistries were found to be normal . Arterial blood gas analysis and echocardiography showed no important abnormalities . Pulmonary function tests ( PFT ) showed a mild restrictive ventilatory defect , with a reduced total lung capacity of 79 % ( 5.94 L ) , forced vital capacity of 80 % ( 4.18 L ) and a forced expiratory volume in one second of 83 % ( 3.72 L ) . The sputum was negative for acid - alcohol resistant bacillus and human immunodeficiency virus testing was negative as well . The chest plain films revealed a diffuse symmetric dense bilateral micronodular pattern ( Figure 1 ) . Based on this finding , HRCT scan was obtained , revealing diffuse ground glass attenuation and septal thickening , more pronounced in lower pulmonary regions , with calcifications along the interlobar septa and subpleural regions . Subpleural cysts were also noticed ( Figure 2 ) . The patient underwent a fiberoptic bronchoscopy with bronchoalveolar lavage and transbronchial lung biopsy . The lavage fluid was negative for tuberculosis or fungi . Microliths were not found . Histology revealed round , concentrically laminated microliths in the alveoli associated with slightly thickened interstitial septa , consistent with the diagnosis of PAM .


A 20 - year - old male with cystic fibrosis was transferred to Houston Methodist Hospital in January 2013 to be evaluated for lung and kidney transplant . Patients with cystic fibrosis are known to develop chronic lung infections that adapt over time to this unique anatomic niche ( 14 , 15 ) . His complicated medical history included pancreatic insufficiency , liver transplantation in 2004 , steroid - induced diabetes , end - stage renal disease , and testicular cancer . He had a long history of respiratory infections with several multidrug - resistant bacteria , including MRSA . He was treated with ceftaroline at an outside hospital immediately prior to transfer to Houston Methodist Hospital . The patient was periodically hospitalized from January to July 2013 and was treated for recurrent respiratory and catheter - related infections caused by MRSA and multidrug - resistant Pseudomonas aeruginosa . His antibiotic exposure included long treatment courses with various agents , including meropenem , ceftazidime , doxycycline , vancomycin , linezolid , cefepime , ciprofloxacin , and inhaled and systemic colistin and tobramycin . Shortly after being readmitted to our hospital in June 2013 , MRSA was grown from cultures of blood and respiratory specimens . These two isolates were resistant to clindamycin , linezolid , oxacillin , and trimethoprim - sulfamethoxazole and susceptible to minocycline , rifampin , and vancomycin . His blood isolate grew confluently around the ceftaroline Etest strip , yielding an MIC of > 32 mg / liter . Five additional S. aureus respiratory tract isolates were available for further study . All MRSA isolates from this patient had a small - colony - variant ( SCV ) phenotype .


A 73 - year - old woman was initially referred 19 months prior to the current presentation , at which time fluorescein and indocyanine green angiography showed a medium - sized subfoveal classic CNV in the left eye , which had not been previously treated . She did not return for treatment until the current presentation , when she noted decreased vision in the left eye . The best - corrected visual acuity ( BCVA ) was 20/100 . Slit lamp examination showed a small yellowish patch in the perifoveal region and retinal edema . Structural OCT revealed the retinal elevation , subretinal fluid and a hyper - reflective elongated area above RPE indicating type II CNV . Anti - angiogenic treatment with intravitreal aflibercept injections were administered with a treat - and - extend regimen . OCT angiograms ( Fig.1 ) showed noticeable reduction in CNV flow area by 1–2 days post injection , with continued reduction at 1 week and 2 weeks . CNV flow area and vessel density were reduced , probably due to the decreased flow or temporary closure of the smaller anastomoses . Significant re - appearance of CNV was noticeable at 4 weeks after the first injection and again at 6 weeks after the second injection . The vascular pattern of the re - enlarged CNV ( Fig.1A ) was very similar to the initial pretreatment CNV , suggesting that the recurrence may be reopening of original channels rather than growth of new vessels . Comparing the CNV network prior to the 3rd injection to the baseline , it is notable that there were fewer smaller channels , while the larger caliber channels remained . Quantitative measurements from OCT angiography ( Fig.2A ) showed reduction in CNV flow area and flow index over the first 2 weeks with subsequent return . Retinal thickness ( Fig.2B ) showed the fluid resorption over the first 4 weeks in the first treatment cycle continuing at least 2 weeks into the second treatment cycle , at which time no fluid remained ( Fig.1B ) . But fluid re - accumulated under the retina 6 weeks after the 2nd injection . Visual acuity ( Fig.2B ) continued to improve over the 3 treatment cycles .


A 69 - year - old man became aware of the onset of exertional dyspnea in July 2010 . He had a history of smoking for more than 40 years , 20 cigarettes per day . His parents and 1 brother had lung cancer . His past medical history was unremarkable . He had no occupational exposure . Due to the exertional dyspnea , he was admitted to the People 's Hospital of Shunde District for echocardiography and computed tomography pulmonary angiography ( CTPA ) . Echocardiography indicated that he had severe PAH , because his pulmonary arterial pressure ( PAP ) was 104 mmHg , and his right heart was enlarged ( the right ventricle diameter : 57 mm ) . CTPA showed that he had not only PAH , but also a thrombus in the upper lobe artery of the right lung . However , the region in the lung affected by this PE was considered too small to induce such a severe PAH . In addition , he did not have connective tissue disease , drug use , history of chemotherapy , or HIV test ( + ) , so he was diagnosed as IPAH combined with PE . He was treated with warfarin ( 2.5 mg per day with a target of the international normalized ratio [ INR ] between 1.5 and 2.5 ) and slidenafil targeted PAH therapy ( 20 mg twice per day ) . The patient 's dyspnea was relieved slightly after the use of these medications for 1 month and the retested PAP by echocardiography was decreased to 72 mmHg , but all the other symptoms and signs failed to further improve after taking these medications for 6 months . In March 2011 , he went to the Kyorin University affiliated hospital in Japan for further treatment . Right heart catheterization revealed PAP 72/34/47 mmHg , pulmonary capillary wedge pressure ( PCWP ) 17/5/10 mmHg , cardiac output 4.3 L / min , cardiac ejection index 2.36 L / min / m2 , and pulmonary vascular resistance 7.3 Wood units . He was retested for CTPA that showed that the thrombus in the upper lobe artery of the right lung disappeared . He was still diagnosed as having IPAH , so treated with sildenafil ( 20 mg 3 times a day ) and sorafenib ( 400 mg once a day ) targeted PAH therapy and oral warfarin anticoagulation therapy ( 2.5–3.75 mg per day , but the daily dose was adjusted by INR that was between 2 and 3 ) . Disappointingly , his dyspnea was not relieved and the PAP tested by the echocardiography was around 95 mmHg . In September 2011 , he visited the Shanghai Pulmonary hospital in China because of no relief of dyspnea . Arterial blood gas analysis showed that PaO2 was 63 mmHg with oxygen saturation 93 % ; lung function test revealed a mild obstructive dysfunction of the pulmonary ventilation ( FEV1 / FVC 62.06 % , FEV1 % Prediction 80.9 % ) and a severe reduction in the diffusing capacity of the lungs for carbon monoxide ( DLCO 42 % ) ; 6 - minute walk test was 385 m. Chest high - resolution computed tomography ( HRCT ) ( Fig.1A and B ) showed smooth thickening of interlobular septa , diffuse centrilobular nodules , and the enlargement of mediastinal lymph nodes . The right heart catheterization revealed that PAP was 83/34/53 mmHg and PCWP was 14/6/11 mmHg . Acute vasodilator testing was negative . Coronary angiography and CTPA were both normal ; pulmonary ventilation / perfusion ( V’/Q ’ ) lung scan showed a matched ventilation and perfusion defects . Bronchoalveolar lavage showed an elevated percentage ( 65 % ) of hemosiderin - laden macrophages with the Golde score 80 ( the normal range : 0–20 ) . He was still diagnosed as having IPAH , but he was suspected as actually having PVOD . He was continuously treated with the targeted PAH therapy , and he was asked to continue his anticoagulation treatment , but he stopped warfarin anticoagulation treatment by himself sometime in 2014 ( he failed to recall the exact date . ) because he felt that it was too cumbersome to frequently test the INR for blood coagulation monitoring . Furthermore , after he discontinued his warfarin therapy , he also failed to monitor D - Dimer to prevent PE recurrence . His dyspnea was relatively stable , but he developed many , but not fatal , episodes of pulmonary edema during the therapy . In January 2015 , he was rushed to hospital because his dyspnea was suddenly exacerbated . CTPA showed a thrombus in the right main pulmonary artery ( Fig.1C ) . Rivaroxaban was administered for anticoagulation treatment , but this medication did not relieve his dyspnea , therefore , he was too ill to walk . Instead , he was bound to his wheelchair for half a year . The severe PAH ( suspected as PVOD ) combined with a life - threatening PE mandates a lung transplantation for his survival . Six months later , he was undergone a bilateral sequential allogenic lung transplantation assisted by veno - arterial extra - corporeal membrane oxygenation ( VA - ECMO ) after he signed the written consent on July 29 , 2015 . Histology of the explanted lung specimen showed pulmonary vein occlusion ( Fig.1D – F ) and the pulmonary embolism in the right pulmonary artery . Until then , he was confirmed as having PVOD combined with PE , instead of IPAH . He has been living well without dyspnea for more than 1 year after the lung transplantation and the echocardiography showed the normalizations of PAP and the once enlarged right ventricle of his heart . This study was approved by the Ethics Committee of The People 's Hospital of Zhongshan City . The written informed consent was obtained from the patient .


A 19 - year - old man presented at the emergency department , 12 h after insertion of a high pressure container with tanning spray into his rectum . A plain abdominal radiograph ( Figure 1 ) showed the container in the rectosigmoid region . There were no signs of perforation . A flexible sigmoidoscopy was performed under conscious sedation . The object was located just above the rectosigmoid junction . The container could not be extracted by bimanual manipulation . An attempt to remove the object with conventional endoscopic instruments , such as polypectomy snares , was unsuccessful . The sigmoidoscope could be passed alongside the foreign body to its proximal end . A guide wire was left behind with the sigmoidoscope removed . Subsequently , a 40 mm pneumatic dilatation balloon ( Rigiflex ® , Boston Scientific ) , normally used in achalasia patients , was inserted over the guide wire and inflated just above the container ( Figure 2 ) . For safety purposes , the sigmoidoscope was reintroduced alongside the catheter of the balloon to allow endoscopic visual control of the distal end of the container in the rectum . Gentle traction was exerted on the balloon catheter , and the container was successfully removed under fluoroscopic and endoscopic control ( Figure 3 ) .


We present the case of a 22 - year - old woman who was diagnosed in childhood with type IIb VHL , with known multiple retinal angiomas , pancreatic cysts , and spinal and cerebellar hemangioblastomas . She was admitted to our hospital complaining of progressive dyspnea and palpitations . On examination , her blood pressure was 159/119 mm Hg and her pulse was 123 bpm . Physical examination revealed a systolic murmur at the mitral focus with irradiation to the axilla and pulmonary rales . Electrocardiography revealed no remarkable findings , except for sinus tachycardia . Laboratory tests showed an elevated N - terminal pro - brain natriuretic peptide and chest X - ray showed signs of pulmonary interstitial edema . The patient was admitted to the Department of Cardiology with the diagnosis of acute decompensated heart failure . Transthoracic echocardiography showed a dilated left ventricle with severely depressed ejection fraction due to generalized hypokinesia and a mild mitral regurgitation ( fig.1a ) . During admission , the patient had several episodes of paroxysmal dyspnea despite treatment with β - blockers and diuretics . Due to the clinical suspicion of adrenal gland disease , 24 - hour urinary catecholamine and metanephrine tests were requested . Cardiac MRI was performed in order to complete the cardiomyopathy study , and confirmed the echocardiographic findings as well as revealed an incidental finding of a great left renal mass . The 24 - hour urine laboratory tests showed markedly elevated levels of norepinephrine , total catecholamines , normetanephrine and metanephrine with normal epinephrine values : 24 - hour norepinephrine 468 μg ( normal range : 23 - 105 ) , 24 - hour epinephrine 13 μg ( normal range : 4 - 20 ) , 24 - hour total catecholamines 658 μg ( normal range : 217 - 575 ) , 24 - hour normetanephrine 2,988 μg ( normal range : 105 - 354 ) , 24 - hour total metanephrine 3,013 μg ( normal range : 0 - 1,000 ) . Therefore , an abdominal MRI was performed , which showed the presence of two cystic masses ( fig.2 ) located at the left hypochondrium . Surgical resection of both masses was performed ( after α - blockade with phenoxybenzamine and β - blockade with propranolol ) through a laparoscopic approach , confirming the diagnosis of pheochromocytoma and clear cell renal carcinoma on histology . During follow - up , blood pressure and heart rate values normalized ( 124/77 mm Hg , 78 bpm ) , 24 - hour urinary catecholamine values returned to normal range ( norepinephrine 69 μg/24 h , total catecholamine 221 μg/24 h , normetanephrine 202 μg/24 h and total metanephrine 259 μg/24 h ) and the patient had no new episodes of palpitations or dyspnea . Six months after surgical resection , echocardiography was repeated and showed a left ventricle with normal diameters and preserved ejection fraction ( fig.1b ) . Genetic analysis revealed a germline mutation ( exon 3 deletion ) of the VHL tumor suppressor gene on the short arm of chromosome 3 . As the patient had no family history of VHL , it was concluded that it was a de novo mutation .


A 34 - year - old Hispanic male without significant past medical history presented to our hospital with a one month history of cough ; productive of whitish sputum . At presentation , he denied fever , chills , night sweats , chest pain , hemoptysis , back pain , recent travel or sick contacts . He reported that he had approximately a 15–20 pound weight loss during the last six months . He denied smoking , socially drank alcohol , and had unprotected sexual intercourse with multiple partners in the past . On admission , his temperature was 97.9 ° F ; heart rate was 85 beats per minute , respiratory rate was 16 breaths per minute , blood pressure was 107/66 mm Hg , and oxygen saturation was 100 % on room air . Examination showed oral thrush , decreased breath sounds and crackles on the right lower lung base . No cutaneous lesions were reported and the rest of clinical examination was unremarkable . His complete blood count ( CBC ) showed hemoglobin of 9.7 g / dL , WBCs 2.3×109 / L , and platelets of 164×109 / L. His creatinine was 0.62 mg / dL , and blood urea nitrogen was 10 mg / dL. Radiograph of the chest showed extensive right and left perihilar opacity more on the right side , and computed tomography ( CT ) scan of the chest showed a right sided large perihilar mass ( Figure 1 ) with multiple thoracic and lumbar vertebrae , ribs , and sternal tiny lytic lesions consistent with bony metastasis ( Figure 2 ) . Blood and sputum cultures were negative . Tuberculosis was ruled out by three consecutive negative sputum smears for acid fast bacilli and a negative QUANTIferon gold test . He tested positive for human immunodeficiency virus ( HIV ) and his CD4 counts came back at 7 cells / uL. He was started on prophylaxis with bactrim 80–160 mg daily and azithromycin 1200 mg weekly for opportunistic infections . Bronchoscopy with biopsy was performed but was unrevealing and he underwent a video assisted mediastinoscopy with biopsies of the right hilar mass . Pathology showed spindle cells positive for CD34 , BCL2 , vimentin , and HHV-8 with diffuse positivity for CD31 diagnostic of KS . He started treatment with HAART in the form of emtricitabine and tenofovir disoproxil fumarate 200/300 mg and dolutegravir 50 mg . The patient was actively involved in decisions regarding management options . He favored HAART isolated regimen without additional chemotherapy . His condition showed continuing clinical improvement ; a repeat CT scan of the chest at three months showed profound regression of the disease with disappearance of most of the lesions ( Figure 3 ) .


In March 2009 , a 21 - year - old man was admitted to another institution with symptoms of intermittent fever , headache , polyarthralgias , skin rash over the trunk , and petechiae in the fingers and palms . The patient was previously healthy , had no history of drug abuse , and took no regular medication . He also had no pets and had not traveled recently . He had been in his usual state of health until one month before admission , when intermittent high fever developed ( maximum axillary temperature , > 39 ° C ) . In addition , he reported headaches , bilateral and symmetric arthralgia on the wrists and hands ( with an inflammatory pattern ) , petechiae over the palms and fingers , salmon - colored rash on the trunk , nausea , and vomiting . An evanescent , salmon - colored rash was observed on his chest and abdomen , and 2 - mm petechiae covered the palms and the fingers . No signs suggested arthritis , and there was no lymphadenopathy or hepatosplenomegaly . Blood testing showed elevated inflammatory markers ( Table II ) . The electrocardiographic ( ECG ) and chest radiographic results were normal . Ibuprofen ( 1,200 mg / d ) and acetaminophen ( as needed ) were begun and provided symptomatic relief . An extensive evaluation was performed , including blood cultures , viral and bacterial serologic studies , immunologic screening ( rheumatoid factor and antinuclear antibodies ) , and computed tomographic ( CT ) studies of the chest , abdomen , pelvis , and cranium . No significant changes were found . However , a transthoracic echocardiogram ( TTE ) showed circumferential , mild pericardial effusion ( maximal diameter , 8 mm ) , with no signs of tamponade or other relevant findings . A few days later , dyspnea and diffuse chest pain developed . The pain was relieved by chest anteflexion and aggravated by deep inspiration and by lying supine . The patient was hypotensive , tachycardic , and hyperpneic , and he manifested jugular venous distention . The ECG showed sinus tachycardia and diffuse ST - segment elevation ( Fig.1 ) , the chest radiograph revealed an enlarged cardiac silhouette ( Fig.2 ) , and the inflammatory markers were again elevated ( Table II ) . A repeat TTE showed an increase in the pericardial effusion ( maximal diameter , 12 mm ) ( Fig.3 ) and collapse of the right heart chambers . Emergency pericardiocentesis drained 60 mL of serosanguineous fluid . When repeated , CT of the chest and abdomen revealed mild right and severe left pleural effusion , as well as mild ascites . Thoracocentesis was then performed , during which 350 mL of serosanguineous pleural fluid was drained . Both heart and lung fluids were exudates that yielded negative microbiologic examination and were unremarkable upon histologic analysis . The diagnosis of AOSD was established , and the patient was medicated with prednisone ( 0.5 mg / kg / d ) , with major clinical and laboratory improvement ( Table II ) . There was also ST - segment normalization , and the follow - up echocardiogram showed only mild pericardial effusion ( 2–3 mm ) , without hemodynamic compromise . Screening for autoimmune and infectious disorders was repeated , and no significant changes were found . The patient was discharged from the hospital on a regimen of prednisone . There was a recurrence of pericardial and pleural effusion 2 months after discharge ( after corticosteroid weaning and withdrawal ) , which was resolved by resuming the prednisone therapy . Ten months after discharge , the patient presented at our institution for a follow - up visit . He was asymptomatic and was maintained on corticosteroid therapy . Follow - up ECG and TTE showed no significant changes ( Figs.4 and 5 ) .


A 74 - year - old man was referred to our hospital in November 2000 because of liver dysfunction detected during a medical checkup . The patient had been diagnosed with nephrotic syndrome in 1995 . Laboratory examinations showed elevated serum hepatobiliary enzymes and IgM , and the presence of antimitochondrial antibodies . Serologic markers for Hepatitis B and C viruses were negative . Histopathologic examination of a liver biopsy specimen obtained at laparoscopy revealed non - suppurative destructive cholangitis in the portal area ( Figure 1 ) . The diagnosis of PBC ( Scheuer stage 3 ) was confirmed and ursodeoxycholic acid , 900 mg daily , was started . In January and June 2002 , the patient underwent endoscopic variceal ligation plus endoscopic injection sclerotherapy as well as argon plasma coagulation for worsening esophageal varices . In September 2007 , the patient was admitted for the treatment of recurrent esophageal varices . The platelet count had ranged between 52 × 109 / L and 69 × 109 / L for several years , but it was noted to decrease from 61 × 109 / L in June 2007 to 8 × 109 / L just before admission . Before the deterioration of thrombocytopenia , the patient had no infectious diseases and received no other medication . On admission , the patient had neither purpura nor bleeding episodes . Table 1 shows the laboratory data on admission . The platelet - associated IgG level was markedly high . Bone marrow biopsy revealed normocellular marrow without cellular atypia . Ultrasonography and magnetic resonance imaging revealed a cirrhotic liver with splenomegaly , ascites , and gallstones . The spleen size had remained unchanged from previous imaging examinations . Based on these findings , the association of PBC ( decompensated liver cirrhosis ) with ITP was diagnosed . Human leukocyte antigen ( HLA ) genotyping determined by polymerase chain reaction - sequencing - based typing or polymerase chain reaction - sequence specific primers ( SRL , Inc. , Tokyo , Japan ) detected A*02010101 , B*400201 , C*030401 , C*07020101 , DPB1 * 0501 , DQA1 * 0103 , DQA1 * 030101 , DQB1 * 030201 , DQB1 * 060101 , DRB1 * 080201 , and DRB1 * 080302 . The 13C urea breath test for H pylori infection was negative . Figure 2 shows the clinical course . Oral prednisolone , 30 mg daily , for ITP was started on day 11 , and diuretic therapy combined with albumin infusion for ascites was performed . As the platelet count did not increase notably , pulse therapy with intravenous methylprednisolone , 1 g daily , was added on d 22 to 24 . However , the response was weak and temporary . On d 31 , mild melena was identified . The patient was given a trial of intravenous immune gamma globulin therapy , 25 g daily , on d 32 to 36 , combined with a second round of intravenous methylprednisolone pulse therapy on d 32 to 34 . Because a moderate response was observed , prednisolone was continued , and the platelet count increased slowly . The ascites was relatively well controlled with diuretics at discharge . Considering the decompensated liver cirrhosis and the platelet count , we determined the patient required careful follow - up of esophageal varices without prophylactic endoscopic therapy .


The patient was a 38 - year - old Caucasian man of Spanish origin . His height was 165 cm and his parents were cousins . Both parents were of normal height and neither showed any minor anomalies of the EvC syndrome spectrum . The patient had two healthy sisters . His morphological features were : ( a ) normal mouth opening with missing lower incisors ( Fig.1 ) ; ( b ) small chest , as determined by a cardiothoracic examination ; and ( c ) disproportionately short extremities with one additional postaxial digit on each hand ( Fig.2 ) . Although no sensory - motor deficit was noted , the patient presented with mild mental retardation . Genetic analysis in his early infancy showed a previously reported homozygous nonsense mutation c.1195 C1 T , p. Arg399X of EvC2 , resulting in loss of function of the protein.(5 ) His parents were heterozygous carriers of the mutation . In 1998 , the patient underwent successful ostium primum atrial septal defect closure through a midsternotomy . He re - presented with embolic cerebral ictus in 2007 , and cavotricuspid isthmus ablation for common atrial flutter was performed successfully . There were no serious aftereffects . During a routine examination in 2012 , a high - pitched systolic murmur was discovered . Electrocardiography showed sinus rhythm with hemiblock and right bundle branch block , while echocardiography revealed a dilated and hypertrophic left ventricle . These abnormalities produced a flow that was directed to the left atrial appendage , leading to severe mitral valve regurgitation . The patient ’s systolic pulmonary artery pressure was 50 mmHg and his aortic valve was normal . His medical treatment included ramipril , duloxetine , alprazolam and acenocumarol . The patient was scheduled for mitral valve surgery . In the operating theatre , the patient ’s right femoral artery was cannulated . A right thoracotomy was performed on the fourth intercostal space and bicaval cannulation was established . Both veins were excluded . The operation used a normothermic cardiopulmonary bypass without cross - clamping of the aorta in the beating heart . To avoid air embolisms , continuous carbon dioxide ( CO2 ) field flooding was applied using a CarbonAid CO2 diffuser ( Cardia Innovation AB , Stockholm , Sweden ) . The attempt to perform a mitral valve repair was unsuccessful and a bileaflet mechanical valve ( no.29 ) was implanted . No complications associated with anaesthesia occurred . The patient ’s postoperative recovery was uneventful and he was discharged on postoperative day 10 . After two years of follow - up , echocardiography revealed optimal function of the mitral valve and a decrease in systolic pulmonary artery pressure ( 30 mmHg ) .


The patient , a 4 - year - old boy , was diagnosed as having Blau syndrome based on the manifestation of typical clinical features ( ankle and wrist arthritis / tenosynovitis , diffuse eczematous rash , and uveitis ) , histologic evidence of noncaseating granulomas , and a heterozygous NOD2 mutation ( p. R334W ) . Ocular involvement was initially controlled by topical and oral corticosteroids , but over the years visual impairment progressed . Other manifestations of Blau syndrome ( arthritis and rash ) subsided over time . Bilateral panuveitis progressed after age 5 years , and was initially treated with methotrexate . However , ocular inflammation persisted despite the addition of local steroid injections and repeated intravenous ( IV ) bolus methylprednisolone treatment ; therefore , when the patient was age 10 years , infliximab ( initially at 5 mg / kg increased to 10 mg / kg IV every 4 to 6 weeks ) was initiated . Although there was an initial improvement , 1 year after this treatment was started uveitis worsened , and at age 12 years infliximab was discontinued . Adalimumab ( 24 mg / m2 every 2 weeks ) was then initiated and the dosage of methotrexate ( 15 mg / m2 / weekly ) was increased . However , ocular disease remained active . Mycophenolate mofetil ( 750 mg / m2 ) and then abatacept ( 10 mg / kg / month IV ) were sequentially administered , without significant improvement . At age 16 years the patient still had granulomatous retinal lesions and anterior chamber inflammation , and macular edema developed , which led to retinal detachment . In addition to the other steroid therapy , corticosteroid pulse therapy was necessary to control disease flares , with an average of 3 boluses / month for 6 consecutive months . Because of the supposed autoinflammatory nature of Blau syndrome , we initiated a trial of IL-1 antibody administration ( 2 mg / kg / month of canakinumab ) . During the 6 months that followed , no ocular flare occurred and no steroid pulse therapy was necessary . Concomitant treatment with oral methotrexate and low - dose prednisone ( 0.2 mg / kg / day ) remained unchanged . Figure 1 shows fluorangiograms before treatment and after the first 6 injections . The drug was well tolerated with no side effects , and findings on laboratory tests ( performed monthly ) were normal .


An 18 - year - old lady presented with history of progressive swaying while walking , worse in night since 5 years . Speech was normal and there was not any tremulousness of upper limbs . Also , there was no history of weakness or numbness . The family history was significant as her elder sister had similar difficulty in walking of 7 - year duration and died because of poorly controlled diabetes mellitus at an age of 20 years . No other members of the kin were affected and there was no history of consanguinity in the parents . The cognitive function was normal and her Mini Mental Status Score was 30/30 . Visual acuity and fundus examination were normal . The extraocular movements including saccades , pursuits were normal , and no saccadic intrusions or nystagmus were noticed . The tone and power were normal in all four limbs ; however , there was generalised exaggeration of myotatic reflexes along with bilateral extensor plantar response . Vibration and joint position sense were intact but Romberg 's was positive . Patient swayed to either side while performing tandem walk . Fasting and postprandial blood glucose levels were normal . Haemogram , renal , liver and thyroid function tests were normal . Serum vitamin B12 and folic acid levels were within normal limits . Nerve conduction study showed absent sensory nerve action potential with normal distal latency , conduction velocity and amplitude of compound muscle action potential ( CMAP ) in all nerves trunks . MRI showed marked atrophy of the cervical cord as compared to cerebellum which was normal ( figure 1 ) . The genetic testing using PCR disclosed expansion of GAA repeat in both alleles ( 254 and 298 ) of FXN gene . A variety of causes of autosomal recessive ataxias need to be considered in the differential diagnosis of FRDA . These were systemically excluded here . Ataxia with vitamin E deficiency ( AVED ) has a phenotype quite similar to FRDA , though titubation and hyperkinesia are more common in AVED.4 A strong family history of ataxia and diabetes mellitus in elder sister favoured FRDA . Ataxia with oculomotor apraxia ( AOA ) types 1 and 2 were not considered owing to lack of apraxia of ocular movements , distal amyotrophy ( severe axonal sensorimotor polyneuropathy ) , atrophy of cerebellum and involuntary movements.5 Ataxia telengiectesia has presentation very similar to AOA types 1 and 2 . Muco - cutaneous markers , sino - pulmonary infections , hypogammaglobulinaemia and radiosensitivity along with predisposition to development of a variety of neoplasms are features of ataxia telengiectasia not seen in AOAs and FRDA . The other important causes of autosomal recessive spastic ataxia apart from FRDA would be autosomal recessive spastic ataxia of Charlevoix - Saguenay and Marinesco - Sjögren syndrome . Charcot - Marie - Tooth disease , an inherited polyneuropathy has gait ataxia in common with FRDA ; however , in this case , CMT was improbable due to following features in this case : lack of motor weakness , retained reflexes , bilaterally extensor plantar and pure sensory neuropathy on nerve conduction study . Idebenone was started at a dose of 450 mg twice a day for its antioxidant properties . For gait ataxia , physiotherapy — Frenkel 's exercise — was initiated . Blood glucose is monitored regularly . At 4 months , there was no significant change in the gait difficulty .


A 58 - year - old man with a past medical history of poorly controlled hypertension and type 2 diabetes , presented to the ICU for a cardiogenic shock complicating acute myocardial infarction . Physical examination revealed reduced level of consciousness ( Glasgow Coma Scale 10/15 ) and weak vital signs ; a blood pressure of 80/50 mmHg , 80 % of oxygen saturation , capillary blood glucose at 2.26 g / L , a heart rate over 125 bpm , and crackling in pulmonary auscultation . Cardiovascular examination showed neither cardiac murmur nor signs of right heart failure . ECG on admission showed normal sinus rhythm , with heart rate of 125 bpm and extended ST - elevation in anterior territory . Laboratory results demonstrated Troponin I level of 6.41 ng / ml , creatinine kinase ( CKMB ) was 67 UI / L , Lactate deshydrogenase was 281 UI / L , glucose level 2.70 g / l , urea 0.40 g / l and creatinine 18.6 mg / L. The patient was intubated and sedated , inotropic agents were started ( norepinephrine 0.4µg / kg / min and dobutamine 20µg / kg / min ) . Chest X - rays showed diffuse alveolar syndrome . Transthoracic echocardiography revealed wall motion abnormalities namely extensive akinesis of anteroseptal , anterior , lateral and inferior walls , and severe left ventricular systolic dysfunction ( ejection fraction of 29 % ) . Medical management was initiated ; anticoagulant therapy for acute coronary syndrome was started ( 500 mg of acetylsalicylic acid and subcutaneous low - molecular - weight heparin ( 0.6ml of enoxaparin ) ) and patient was prepared for myocardial revascularization by coronary angioplasty . Because of non - improvement of neurological status and occurrence of seizures , a brain CT was indicated and revealed infratentorial diffuse hemorrhage ( Fisher grade III ) ( Figure 1 ) . Cerebral angiography confirmed a dissecting aneurysm of an anastomotic branch between left PICA and the V4 segment of left vertebral artery Figure 2 that was successfully embolized . After 24 hours , the patient improved with withdrawal of vasoactive drugs in 24 hours . Left hemicorporeal seizures persisted despite anticonvulsant treatment . Control brain CT did not report rebleeding and angiography showed complete exclusion of the aneurysm while the EEG revealed a diffuse brain damage . 10 days after admission , the patient was discharged in stable condition but still suffers from amnesia .


A 57 - year - old male was admitted to our hospital with 5 months ’ history of massive rectal bleeding ( rectorrhagia ) . He had past history of passage of fresh blood mixed stool since last 55 years . He first complained of rectal bleeding at the age of 2 . He went hospital numerous times for this symptom ; multiple interventions were done but disease was not cured completely . During one of his hospital visit in the past ( patient forgot the date ) , he was diagnosed and treated as hemorrhoids . The symptom was relieved for a while and then recurred again . Thirty years ago , he was diagnosed as rectal hemangioma and managed with cryotherapy in our hospital . That could also just relieve the symptom for few years and it recurred again . Then , he consulted many other hospitals , but could only get short - term symptomatic relief without proper treatment of the cause . Since 5 months , the severity of rectal bleeding increased . He then went to local hospital where intervention was done to control bleeding and symptomatic treatment was done with intravenous fluid and blood transfusion . He finally referred to our hospital for further evaluation . Intermediate rectal bleeding of fresh blood was presented on admission . Patient complained of dizziness on standing , shortness of breath , and palpitation on walking a short distance . There was no history of similar illness in family . On general examination , patient appeared pale , but his heart rate and blood pressure were within normal limit during rest . He lost 5 kg of his body weight during last 5 months . On rectal examination , fresh blood was seen around anal region and soft mass was felt on digital rectal examination . On proctoscopy , anal cavity and rectum were seen filled with fresh blood , but active site of bleeding , polyp , or ulcer was not detected . On laboratory examination , red blood cell count was 3.09 × 1012 / L ( Normal : 4.32–5.72 × 1012 / L ) and hemoglobin was 86 g / L ( Normal : 135–175 g / L ) . All other parameters were within normal limit . None enhanced CT showed isodense ( 35 HU ) homogenous bowel wall thickening that on contrast - enhanced CT venous phase enhances heterogeneously . Multiple calcifications were seen in the thickened bowel wall and around the peri - rectal area . Lesion was seen extending from distal sigmoid to whole of the rectum ( Fig.1 ) . Multiple hypodense lesions were also seen in spleen ( Fig.2 ) . After initial management of anemia , the patient underwent abdominal laparotomy followed by surgical excision . During surgery , 25 cm long lesion was found extending from distal sigmoid to whole of the rectum . Whole of the rectum and part of the sigmoid colon were excised and sigmoid - anus anastomosis was done . Postsurgical histopathological examination of excised specimen showed submucosal multiple thin - walled vessel of varying size with interposed stroma . Some vessels lumen consisted of blood cells ( consistent with blood vessel ) , whereas other consisted of clear fluid ( consistent with lymph vessel ) . Immunohistochemistry of specimen showed endothelial cells positive for CD 31 and CD 34 . Some cells were positive for D2–40 , while others were negative for D2–40 ( Fig.3 ) . On the basis of histopathological report and immunohistochemistry , hemolymphangioma was diagnosed . The surgery , which followed by complication ( intestinal infection ) , was well managed and the patient was discharged from hospital on the 23rd day of surgery . Then after , no further complication or recurrence was noticed during 6 months ’ follow - up . This study was approved by the First Affiliated Hospital of Sun Yat - Sen University Institutional Review Board . Written consent for this case report was obtained from the patient .


A 53 year old female without significant past medical history developed severe viral pneumonia , with rapid , progressive deterioration in her respiratory status . She developed ARDS and mechanical ventilatory management using ARDS protocol were unable to maintain adequate oxygenation . As a result , bedside VV - ECMO was planned . Transesophageal echocardiography ( TEE ) was performed to visualize proper positioning of the guidewire and cannula . Using the Seldinger technique , the right internal jugular vein was accessed and a guide wire was placed . Placement of the guidewire into the IVC proved difficult due to repeated migration of the guidewire into the right ventricle . After multiple attempts , the guidewire was visualized to course properly from the SVC to the IVC . After a bolus dose of 5000 units of intravenous heparin was given , the right internal jugular venous access site was dilated . Just as the final dilatation was completed and upon dilator exchange with simultaneous advancement of the 23 French Avalon cannula , TEE lost visualization of the guidewire . Multiple premature ventricular beats were noted and immediately , a new , rapidly enlarging pericardial effusion was detected ( Figure (Figure2).2 ) . Emergent preparations were made for bedside surgical decompression of the pericardial space . Quickly the patient lost blood pressure from acute cardiac tamponade . The Avalon cannula was immediately clamped at the end but not removed . A emergent subxiphoid pericardial window was performed , resulting in drainage of venous blood and restoration of blood pressure . Transfusion was initiated and the patient was emergently transported to the operating room for surgical exploration . The Avalon cannula was found to have perforated the apex of the right ventricle . The injury was repaired primarily and the Avalon cannula was repositioned toward the IVC again by TEE with additional direct manipulation . VV - ECMO was initiated and the oxygenation improved . Due to excessive coagulopathies , the sternum was left open but was closed on postoperative day 2 . From that point , she remained free from any cardiac or infectious complications and her pulmonary condition slowly improved . She was successfully weaned from VV - ECMO on postoperative day 9 and was discharged home on postoperative day 24 without the need for home oxygen . She regained full physical functions at home and recovered normal pulmonary function by 3 months following discharge from the hospital .


A 75 - year - old man was referred to our hospital for the evaluation of dysphagia for 9 months . He had associated symptoms of nausea and vomiting but denied hematemesis , hematochezia , lethargy , and dyspnea . He was an active smoker and consumed alcohol for 30 years . Physical examination revealed no peripheral lymphadenopathy , thyromegaly , ascites , or mass in the abdomen . Laboratory data were normal : HBs - Ag negative , HIV negative , and syphilis negative . Test for serum antibody against Helicobacter pylori was negative . Chest computed tomography ( CT ) was notable for a huge well - circumscribed and homogeneous cylindrical mesenchymal neoplasm measuring 15.5×5.9×4.0 cm in lower and middle esophagus with mild contrast enhancement ( Fig.1A ) . CT revealed no abnormalities in the lungs , heart , ribs , or mediastinum . Endoscopic examination showed a submucosal lesion in the esophagus starting at 20 cm from the incisor teeth extending up to the cardia ( Fig.1B ) . Endoscopic ultrasonography ( EUS ) revealed hypoechoic lesion with a clear boundary located in the 4th layer . The mass appeared as a benign tumor and was preoperatively diagnosed as esophageal leiomyoma based on chest CT , endoscopic examination , and EUS findings . Endoscopic mucosal resection or endoscopic submucosal resection was not possible because of large size . Hence , surgical resection was planned and informed written consent was taken . The patient underwent thoracoscopic - assisted resection of the mass with gastroesophageal anastomosis , thoracic duct ligation , and jejunostomy . Postoperative course was uneventful . Grossly , a spindle shaped lump measuring 14 × 3.5 × 2.5 cm , was observed in the resected esophagus , which grew into the esophageal lumen and blocked most of lumen . This lump located in the submucosa was covered with intact mucosa , without erosion , ulcer , and hemorrhage . Its cut surface was homogenously white to grayish - white in color . Histological examination using hematoxylin and eosin staining revealed that tumor was covered with intact squamous epithelium , arising from the submucosal layer and expanding in to the muscular layer . The tumor was composed of many nodules of varying sizes separated by collagen fibers . Numerous cytoplasm - rich cells were observed in the collagenous septations with invasive growth pattern ( Fig.2A – C ) . The nodules were mainly composed of small to mid - sized centrocyte - like or monocyte - like cells arranged in diffuse pattern . These atypical lymphocytes possessed clear boundary , pale cytoplasm , irregular nucleus , and occasional nucleolus ( Fig.2D ) . Mitosis was rare . No lymphoepithelial lesion was recognized in the lesion . On immunohistochemical staining , epithelium was diffusely positive for cytokeratin ( Fig.3A ) , and the tumor cells were diffusely positive for cluster of differentiation ( CD)20 , paired box 5 ( Fig.3B ) , and B - cell lymphoma ( Bcl)-2 ( Fig.3D ) . Small deposits of tumor cells , which were distributed mainly in the collagen fibers , were positive for multiple myeloma oncogene 1 , CD138 , and CD43 . Several tumor cells were also positive for CD30 . Small lymphocytes were positive for CD3 and CD5 . Follicular dendritic cells were positive for CD21 and CD23 ( Fig.3C ) . All cells were negative for CyclinD1 , CD10 , Bcl-6 , and B lymphocyte specific activation of OCT binding protein 1 . These follicular dendritic cells were arranged in nodules in which the tumor cells were relatively evenly distributed . This pattern was suggestive of follicular colonizations seen in MALT lymphoma . In this case , gene rearragements and clonality analysis of immunoglobulin heavy chain gene , kappa light chain gene , and lambda light chain gene was performed using the IdentiCloneTM IGH / IGK / IGL Gene Clonality Assay ( InVivoScribe Technologies , CA ) . On monoclonal gene rearrangement , 1 band appeared within 150 to 175 base pairs ( bp ) in heavy chain gene , 2 discrete bands within 225 to 250 bp in kappa light chain gene , and 1 band within 125 to 150 bp in lambda light chain gene ( Fig.4 ) . In addition , no Epstein – Barr virus was observed in this lymphoma on in situ hybridization using the EB virus - encoded small RNA ( Fig.3E , F ) . Based on the clinical data , pathological , immunohistochemical , and gene rearragements analysis , final diagnosis of primary esophageal MALT lymphoma was made . After the surgical resection , no additional therapy in the form of chemotherapy or radiotherapy was administered . Over the 8 months of follow - up , no evidence of recurrence or metastases was found on CT and the patient has been asymptomatic . Ethical approval for this study was obtained from Medical Ethics Committee of the Third Affiliated Hospital , the Third Military Medical University . Written informed consent was obtained from the patient for the publication of this case report and the accompanying images .


This case report was approved by the Review Board of Shandong Power Central Hospital and the patient 's parents signed informed consent for publication of the case report and any accompanying images . A 4 - year - old female child presented at the outpatients department with slowly enlarging , painless , symmetrical swelling of both cheeks . When the girl was approximately 2 years old , the parents noticed a change in her facial symmetry , which later became more obvious . On palpation , the swellings were bony and hard in consistency . The skin over the swelling was smooth and freely movable , with no increase in temperature ( Fig.1 ) . There was no history of any trauma , pain , pus discharge , blood discharge , fever , paresthesia , anorexia , or weight loss . No significant family history was reported . Panoramic radiography revealed involvement of both the mandible and the maxilla , and showed a soap bubble - like multilocular radiolucency with thin and expanded cortices . Unerupted teeth included : 15 , 25 , 35 , 37 , 45 , and 47 was impacted ( Fig.2 ) . Multislice computed tomography showed the presence of multilocular cystic lesions filled with soft - tissue density material and dental anomalies affecting the body and the sides of the mandible . Similar lesions were also observed in the maxilla ( Fig.3 ) . A biopsy was obtained from the central area of the left rami . Histologic examination showed many large multinucleated giant cells in a loose , delicate fibrillar connective tissue stroma containing large numbers of fibroblasts and many small blood vessels ( Fig.4 ) . As the child grows older , the lesions often become static and may show regression at puberty . In the current case , no surgical intervention was under taken and the patient was followed up at regular intervals .