ABSTRACT
Endocrine tumors of the extrahepatic bile ducts are an extremely rare clinical entity with only very few cases being reported to date. Two additional cases of extrahepatic endocrine tumors are presented and a review of the literature is analyzed to provide a comprehensive data collection. Thirty-four cases of extrahepatic endocrine tumor, including our 2 cases, have been reported; 16 (47%) being carcinoma. The mean age of presentation was 48.2 years with a male/female ratio of 0.36. Jaundice, the most common symptom, was found in 63% of the patients. The common bile duct and the hilar region were the two most frequent anatomic locations. The mean size of resectable tumors was 2.37cm (0.2- 5.5cm). Metastases were present in 11 patients (32.35%) and local invasion was found only in 6 patients (17.6%). Curative surgery was realized in the majority of the patients. Long-term disease-free survival as long as 20 years was reported when surgery was curative. Endocrine tumors of the extrahepatic duct are exceptional and represent 0.2% of all cancers of these anatomic sites. Curative resection is the most important prognostic factor. Therefore, aggressive surgical therapy with curative intention offers the only chance for cure and has to be considered whenever possible.
Subject(s)
Bile Duct Neoplasms/surgery , Carcinoid Tumor/surgery , Liver Neoplasms/secondary , Adult , Aged , Bile Duct Neoplasms/diagnosis , Bile Duct Neoplasms/mortality , Bile Duct Neoplasms/pathology , Bile Ducts, Extrahepatic/pathology , Bile Ducts, Extrahepatic/surgery , Biomarkers, Tumor/analysis , Carcinoid Tumor/diagnosis , Carcinoid Tumor/mortality , Carcinoid Tumor/pathology , Diagnosis, Differential , Diagnostic Imaging , Female , Hepatectomy , Humans , Jaundice, Obstructive/diagnosis , Jaundice, Obstructive/mortality , Jaundice, Obstructive/pathology , Jaundice, Obstructive/surgery , Liver/pathology , Liver Neoplasms/diagnosis , Liver Neoplasms/pathology , Liver Neoplasms/surgery , Lymphatic Metastasis/pathology , Male , Neoplasm Invasiveness/pathology , Survival RateABSTRACT
BACKGROUND/AIMS: The aim of this study was to evaluate the role of surgical treatment in the management of primary and secondary liver endocrine tumors. METHODOLOGY: We reviewed our experience of surgical treatment for primary and secondary liver endocrine tumors in 34 patients from 1980 to 1999. There were 31 cases of liver metastases from distant primary endocrine tumors treated by curative resection in 11 patients, cytoreductive surgery in 15 and orthotopic liver transplantation in 5. The remaining 3 consisted of primary liver endocrine tumor in 2 patients, liver invasion from primary bile duct endocrine tumor in 1, and all of them were treated by curative liver resection. RESULTS: In patients with distant primary endocrine tumors, the actuarial 5-year survival after curative liver resection, cytoreductive surgery, and orthotopic liver transplantation was of 91%, 50% (being 90% for non-functioning tumors), and 60%, respectively. In patients with primary liver endocrine tumors, long-term survival and disease-free survival (52-240 months) was achieved after curative resection. CONCLUSIONS: When indicated, liver surgery can offer cure and/or improve survival in patients with primary or secondary liver endocrine tumors. The choice of the surgical modality should be evaluated carefully.
