Primary hepatic malignant fibrous histiocytoma: diagnostic pitfalls and therapeutic challenge.

BACKGROUND: Due to the rarity of primary hepatic malignant fibrous histiocytoma (MFH), the natural history, optimal management and prognosis are poorly characterized. METHODOLOGY: Between January 2003 and December 2008, we treated 12 consecutive patients with primary hepatic MFH. The patient demographics, tumor characteristics, type of treatment and actuarial survival were analyzed. RESULTS: The mean +/- SD tumor size was 8.4 +/- 3.2cm. Four patients had satellite lesions. R0, R1 and R2 resection of the liver tumor were achieved in 5, 2 and 5 patients, respectively. There was no hospital mortality and the complication rate was 8.3%. At a median follow-up of 11.3 months, local recurrence had occurred in 6 patients and local recurrence + distant metastases in 3 patients. Most patients (8/12) died of the tumor within a year after surgery, with a median survival of 6.1 months. For the remaining 4 patients, 2 patients had undergone surgery for less than 1 year previously, one patient who had a R0 liver resection with extrahepatic metastasis survived for 14 months with multiple metastases, and another patient who had a R0 liver resection but without extrahepatic metastasis survived for 60 months and was disease free. The median survival for the R0 liver resection group carried out in patients without extrahepatic metastases was 8.5 months, while the median survival of the debulking group (R0 liver resection with extrahepatic metastasis/ R1 or R2 liver resection) was 7 months. There was no significant difference in survival between the two groups. CONCLUSION: Hepatic resection was safe for patients with primary MFH with a poor prognosis. Complete resection offers the only hope of long-term disease free survival.

Gallbladder cancer--a comprehensive review.

AIM: Gallbladder cancer is the fifth most common cancer involving the gastrointestinal tract, but it is the most common malignant tumour of the biliary tract worldwide. The percentage of patients diagnosed to have gallbladder cancer after simple cholecystectomy for presumed gallbladder stone disease is 0.5-1.5%. This tumour is traditionally regarded as a highly lethal disease with an overall 5-year survival of less than 5%. The marked improvement in the outcome of patients with gallbladder cancer in the last decade is because of the aggressive radical surgical approach that has been adopted, and improvements in surgical techniques and peri-operative care. This article aims to review the current approach to the management of gallbladder cancer. METHODS: A Medline, PubMed database search was performed to identify articles published from 1990 to 2007 using the keywords 'carcinoma of gallbladder', 'gallbladder cancer', 'gallbladder neoplasm' and 'cholecystectomy'. RESULTS AND CONCLUSIONS: The overall 5-year survival for patients with gallbladder cancer who underwent Ro curative resection was reported to range from 21% to 69%. Laparoscopic cholecystectomy is absolutely contraindicated when gallbladder cancer is known or suspected pre-operatively. Patients with a pre-operative suspicion of gallbladder cancer should undergo open exploration and cholecystectomy after proper pre-operative assessment. For patients whose cancer is an incidental finding on pathological review, a second radical resection is indicated except for Tis and T1a disease. There is still controversy for the optimal management of T1b disease. Although the role of surgery for advanced disease remains controversial, patients with advanced gallbladder cancer can benefit from radical resection, provided a potentially curative Ro resection is possible. There is still no effective adjuvant therapy for gallbladder cancer.

Management of endoscopic retrograde cholangiopancreatography-related perforation.

Endoscopic retrograde cholangiopancreatography (ERCP)-related perforation occurs in 0.1-1.8% of patients after therapeutic ERCP. It is a serious complication with a high mortality. However, little evidence exists in the medical literature and the optimal management remains unclear. This review aims to critically evaluate the optimal management of therapeutic ERCP-related perforation.