Malignant potential of intrahepatic biliary papillomatosis: a case report and review of the literature

Abstract

Background: Biliary papillomatosis (BP) is a rare disease entity with a strong malignant potential. It is characterized by multiple papillary adenomas involving both the intrahepatic and extrahepatic biliary tree. BP was considered in the past to be a disease with low malignant potential. However, a current review of the English literature revealed a high rate of malignant occurrence of approximately 41% and histological analysis along with the expression pattern of mucin core proteins (MUC) and mucin carbohydrate antigens suggests that BP is a borderline or low grade malignant neoplasm with a high malignant potential.

Recurrent cholangitis in a 65-year-old man

“…Despite advances in diagnostic technology distinguishing, this disease from other inflammatory and neoplastic conditions remains challenging 3. Like in our case, patients are often misdiagnosed as having PSC.…”

Rare event of biliary papillomatosis arising in a choledochal cyst

“…BP can involve any part of the biliary system including the gallbladder and pancreas producing large amounts of a thick jelly like substance leading to obstruction to bile flow and recurrent episodes of cholangitis 5. BP is considered to be a benign lesion; however, malignant transformation has been seen in as many as 35–41% cases which include dysplastic changes to carcinoma in situ and to invasive adenocarcinoma 6 – 8. Males are frequent sufferers (male:female=2:1) 8.…”

“…BP is considered to be a benign lesion; however, malignant transformation has been seen in as many as 35–41% cases which include dysplastic changes to carcinoma in situ and to invasive adenocarcinoma 6 – 8. Males are frequent sufferers (male:female=2:1) 8. Median age at diagnosis is 58 years 3.…”

“…Adenocarcinoma arising from BP in a choledochal cyst has been reported in 1 case 3. A classification has been proposed to define the malignant potential of BP: class 1 is BP with low-grade atypia, class 2 with high-grade atypia, class 3 with in situ carcinoma, class 4 with microscopic foci of stromal invasion and class 5 with invasion into hepatic parenchyma or bile duct wall 8. However, this classification may not hold true for BP arising in a choledochal cyst.…”

Multidisciplinary Treatment of Aggressive and Rapidly Progressing Biliary Papillomatosis

“…The natural course of this disease seems to vary considerably [5]. Although malignant transformation occurs in about 50% of cases and the prognosis in general is poor, standard recommendations cannot be given [6]. Described procedures range from local ablation or limited liver resections to orthotopic liver transplantation [7].…”