Clinicopathologic review of 58 patients with biliary papillomatosis.
BACKGROUND
Biliary papillomatosis (BP) is a rare disease that is characterized by multiple numerous papillary adenomas in the biliary tree. The clinical features and outcome, however, are not well known. The authors retrospectively analyzed their clinicopathologic features and long-term follow-up results.


METHODS
Between March 1995 and January 2003, 58 patients were diagnosed with BP by cholangioscopic and histologic findings at a tertiary referral center, Asan Medical Center (University of Ulsan College of Medicine, Seoul, Korea). The authors retrospectively reviewed the medical records to obtain demographic, radiologic, cholangioscopic, and pathologic data.


RESULTS
The common clinical manifestations at the presentation of patients were repeated episodes of abdominal pain, jaundice, and acute cholangitis. Acute cholangitis was more common in patients with mucin-hypersecreting BP (MBP), whereas patients with nonmucin-producing BP (NMBP) were more asymptomatic (P < 0.05). Papillary adenocarcinoma and mucinous carcinoma were detected in 48 patients (83%) with papillary adenomas. Overall survival rates of NMBP and MBP were 89% and 69% at 1 year, 57% and 37% at 3 years, and 52% and 19% at 5 years, respectively. The mean survival period of NMBP and MBP was 52.27 +/- 6.72 months and 30.84 +/- 8.36 months, respectively.


CONCLUSIONS
BP should be regarded as a premalignant disease with high malignant potential. The pathogenesis of progression from benign to malignant disease may follow the adenomacarcinoma sequence. Although clinical presentations were somewhat different for patients with NMBP and MBP, the long-term survival rate was similar.
Lee
Sang Soo
SS
Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
Kim
Myung-Hwan
MH
Lee
Sung Koo
SK
Jang
Se Jin
SJ
Song
Moon-Hee
MH
Kim
Kyu-Pyo
KP
Kim
Hong Ja
HJ
Seo
Dong-Wan
DW
Song
Dong Eun
DE
Yu
Eunsil
E
Lee
Seung-Gyu
SG
Min
Young Il
YI
D015746:Abdominal Pain / Q000209:etiology; D000236:Adenoma / Q000473:pathology*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001661:Biliary Tract Neoplasms / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D010212:Papilloma / Q000473:pathology*; D011230:Precancerous Conditions; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis