Biliary papillomatosis (BP) is rare type of papillomatosis that involves the biliary ducts.
It is often observed in middle-aged and elderly patients and is more common in men (male-to-female ratio 2:1).
Biliary papillomatosis is usually at an advanced stage when first diagnosed. Clinical symptoms include jaundice, repeated episodes of abdominal pain, and acute cholangitis.
It is characterized by multiple papillary adenomas that involve the intra- or extrahepatic biliary tree. It is pathologically classified into 5 classes according to the degree of cytological and structural atypia 3.
Recognised associations include:
- Caroli disease
- choledochal cyst
- polyposis coli
- ulcerative colitis
- cirrhosis related to hepatitis B and C viral infections
Approximately 40% of patients with biliary papillomatosis have a mildly elevated CA 19-9 level.
There are various imaging patterns:
- diffuse involvement
- intraductal mass
Ultrasound may show a nonshadowing solid mass of intermediate echogenicity is shown within the duct. Lesions can be well defined and demarcated from the bile duct wall. Proximal ductal dilatation is usually present. There may be concomitant intrahepatic and extrahepatic duct calculi 5.
Both intra- and extrahepatic duct dilatation may be seen.
Hypoattenuating intraductal soft-tissue masses may be seen both before and after intravenous contrast.
MRI - MRCP
Reported signal characteristic of lesions include
- T1: low signal
- T2: slightly hyperintense
- T1 C+ (Gd): do not significantly enhance and remain hypointense relative to the adjacent liver parenchyma 2,5
- MRCP: may allow direct visualisation of the papillary lesions or an irregular lumen
Characteristic ERCP findings include multiple filling defects and a dilated biliary tree with serrated irregularity of the bile duct wall. Due to obstruction and mucin secretion, the dilated bile ducts may not completely fill with contrast material. Therefore small papillomas may not be observed.
Treatment and prognosis
Although the lesions are considered benign, they have a high potential for malignant transformation. The risk of malignant transformation is thought to range around 20-50%.
Treatment will depend of type of disease.
In diffuse papillomatosis, liver transplantation can be considered.
Imaging differential considerations include:
Biliary papillomatosis was initially described in 1894 by Chappet. In 1959 anatomical description was given by Caroli .4
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