Eight Cases of Mucin-hypersecreting Cholangiocarcinoma Similar to Intraductal Papillary Mucinous Tumor of the Pancreas

Chang, Hye Sook; Kim, Hong Ja; Na, Joo Ock; Lim, Byung Cheol; Yoo, Kyo Sang; Park, Eun Taek; Seo, Dong Wan; Lee, Sung Koo; Kim, Myung Hwan; Min, Young Il

Abstract

BACKGROUND/AIMS: Mucin-hypersecreting cholangiocarcinoma is very similar to intraductal papillary mucinous tumor (IPMT) of the pancreas. But this disease is rare and its clinicopathologic features are not well known. The purposes of this study are to report our experience of eight patients with the tumor and to analyze the characteristics of its clinical, radiological and pathological finding.
METHODS
Eight patients (3 men and 5 women, mean age 52 years) were diagnosed as having a mucin-hypersecreting cholangiocarcinoma among 4,570 cases of ERCP from 1995 to 1998.
RESULTS
Recent or previous attacks of biliary pain or acute cholangitis were elicited in most of the patients. CT showed diffuse intra- and extra-hepatic duct dilatation in all patients and suspicious ill-defined mass shadow in 2 out of 8 patients. On cholangioscopic examination, a papillary mass or minute mucosal lesion was found in the dilated bile duct containing thick viscid mucin. Eight patients were recommended an operation and curative resection was performed in seven patients. Histologically, well-differentiated adenocarcinoma in the background of benign hyperplasia and adenoma was documented in all patients.
CONCLUSIONS
Mucin- hypersecreting cholangiocarcinoma may be characterized by exceedingly similarity to IMPT of the pancreas in clinical, radiological and pathological features. Further investigation is needed for detailed information such as natural history and outcome.