Primary clear cell sarcoma of the ileum: an uncommon and misleading site

A clear cell sarcoma, arising primarily in the ileum of a 35-year-old man, is reported. Histologically, the neoplasm infiltrated the full thickness of the intestinal wall. It consisted of strands and sheets of round to spindle-shaped cells with clear to eosinophilic cytoplasm, vesicular nuclei and prominent nucleoli. Vascular invasion was present at diagnosis. Tumour cells expressed S-100 protein, melan-A and tyrosinase. They were negative for HMB45, CD117, cytokeratins, epithelial membrane antigen, smooth muscle actin, desmin, CD31, CD34, chromogranin and synaptophysin. Reverse transcription-polymerase chain reaction analysis performed on paraffin-embedded tissue showed EWS-ATF1 fusion transcripts representative of the t(12;22) (q13;q12) clear cell sarcoma reciprocal translocation. The patient, who developed liver metastases 2 months after diagnosis, died of disease at 15 months. This case demonstrates that the gastrointestinal tract is a potential site for primary clear cell sarcoma of soft tissues, and, furthermore, that cytogenetics and/or molecular techniques play a central role in the diagnosi

Gallbladder agenesis diagnosed intra-operatively: a case report

<p>Abstract</p> <p>Introduction</p> <p>Agenesis of the gallbladder is a rare congenital anomaly occurring in 13 to 65 people of a population of 100,000. The rarity of the condition, combined with clinical and radiologic features that are indistinguishable from those of more common biliary conditions, means that it is rarely diagnosed preoperatively, and patients undergo unnecessary operative intervention.</p> <p>Case presentation</p> <p>This case report describes the case of a 79-year-old symptomatic Caucasian man who underwent laparoscopic cholecystectomy for suspected choledocholithiasis despite imaging studies raising suspicion of gallbladder agenesis. Intra-operatively, the diagnosis of gallbladder agenesis and associated common bile duct stones was made.</p> <p>Conclusion</p> <p>The preoperative diagnosis of this rare condition is difficult to make. However, with advances in biliary tract imaging and with heightened awareness of this anomaly, fewer patients will need to undergo unnecessary operative intervention. The authors review the different imaging modalities available to help diagnose this condition and highlight the importance of being aware of this rare anomaly to avoid an operation that carries a high risk of iatrogenic injury.</p

Primary clear cell sarcoma of the ileum: an uncommon and misleading site

A clear cell sarcoma, arising primarily in the ileum of a 35-year-old man, is reported. Histologically, the neoplasm infiltrated the full thickness of the intestinal wall. It consisted of strands and sheets of round to spindle-shaped cells with clear to eosinophilic cytoplasm, vesicular nuclei and prominent nucleoli. Vascular invasion was present at diagnosis. Tumour cells expressed S-100 protein, melan-A and tyrosinase. They were negative for HMB45, CD117, cytokeratins, epithelial membrane antigen, smooth muscle actin, desmin, CD31, CD34, chromogranin and synaptophysin. Reverse transcription-polymerase chain reaction analysis performed on paraffin-embedded tissue showed EWS-ATF1 fusion transcripts representative of the t(12;22) (q13;q12) clear cell sarcoma reciprocal translocation. The patient, who developed liver metastases 2 months after diagnosis, died of disease at 15 months. This case demonstrates that the gastrointestinal tract is a potential site for primary clear cell sarcoma of soft tissues, and, furthermore, that cytogenetics and/or molecular techniques play a central role in the diagnosi

High APACHE II score and long length of bowel resection impair the outcomes in patients with necrotic bowel induced hepatic portal venous gas

<p>Abstract</p> <p>Background</p> <p>Hepatic portal venous gas (HPVG) is a rare but potentially lethal condition, especially when it results from intestinal ischemia. Since the literatures regarding the prognostic factors of HPVG are still scarce, we aimed to investigate the risk factor of perioperative mortality in this study.</p> <p>Methods</p> <p>We analyzed data for patients with intestinal ischemia induced HPVG by chart review in our hospital between 2000 and 2007. Factors associated with perioperative mortality were specifically analyzed.</p> <p>Results</p> <p>There were 22 consecutive patients receiving definite bowel resection. 13 cases (59.1%) died after surgical intervention. When analyzing the mortality in patients after bowel resections, high Acute Physiology And Chronic health Evaluation (APACHE) II score (<it>p < 0.01</it>) and longer length of bowel resection (<it>p </it>= 0.047) were significantly associated with mortality in univariate analyses. The complication rate was 66.7% in alive patients after definite bowel resection.</p> <p>Conclusions</p> <p>Bowel resection was the only potential life-saving therapy for patients with mesenteric ischemia induced HPVG. High APACHE II score and severity of underlying necrotic bowel determined the results in patients after bowel resection.</p