Endocrine cancer of the extrahepatic bile duct – case report

Endocrine tumours arising from the extrahepatic biliary ductal system are rare with a 0.2–2% frequency of all extrahepatic neoplasms. At this time, 34 cases of such endocrine tumours had been described. Among these neoplasms, only 17 cases were cancers. The most frequent clinical and biochemical signs of these cancers were jaundice, right subcostal pain and elevation in cholestatic liver enzyme levels. Preoperatively, cholangiography and CT-scan were found to be highly non-specific for the final diagnosis; however, these diagnostic procedures had been preformed in less than 6% of all cases. Percutaneous needle biopsy and endoscopic brush cytology provided the right preoperative diagnosis. With curative surgery the tumour-free survival can be ranged from 5 months up to 20 years, which suggests a more benign clinical course than in cholangiocancer.

Case report: A 63 year-old man with a history of hypertension and coronary artery disease was admitted since jaundice, weight loss and elevated cholestatic liver enzymes. Contrast enhanced CT-scan followed by MR-cholangiography disclosed a hilar space occupying lesion measured 80×70mm in the larger diameters suggesting Bismuth III-IV stage extrahepatic biliary stricture. Because of the high suspicion of malignancy, absence of metastatic disease and the good clinical condition, total resection of the hilar neoplasm could be performed. Immunohistochemical study of the resection specimen showed invasive endocrine tumour, which finding was consistent with the presence of endocrine cancer. After uneventful recovery, the patient has received subsequent chemotherapy and has a 1-year tumour-free survival.

Conclusion: This report adds further insight on the course and response to therapy of extrahepatic endocrine cancers. The relative long term disease-free survival after curative surgery may demonstrate that the nature of these tumours may be less malignant than cancers originated from the biliary epithelium. The possible presence of endocrine tumour should be always considered in the preoperative diagnostic procedures of proximal biliary tree strictures.