Bile duct papillomatosisD37.6

Synonyms

Biliary papillomatosis; biliary papilloma; intraductal papillary neoplasm of the biliary tract (IPNB);

First describer

The first describer of biliary papillomatosis was Chappet in 1894 (Gu 2015).

Biliary papillomatosis (BP) is characterized by multiple papillary adenomas in both the intrahepatic and extrahepatic biliary tree (Hsu 2014).

BP is considered a benign tumor, but represents a precancerous condition (Herold 2022) with definite malignant potential (Hsu 2014).

Histologically, one differentiates between mucin- or non-mucin-secreting papillomas (Hsu 2014). The secreting papillomas so-called intraductal papillary mucinous neoplasms = IPMNB produce mucin, occur singly and multiply. They are found in intra- and extrahepatic bile ducts (Lampichler 2019).

Biliary papillomatosis is a rare disease that occurs predominantly in middle to older age (Hsu 2014). The ratio of males to females is 2: 1 (Gu 2015).

Bile stasis and recurrent infections likely result in chronic inflammation with subsequent mucosal changes, ultimately leading to papillomatosis (Gu 2015).

The pathophysiology is unknown. It is most likely that malignant transformation follows the pathway from adenoma to carcinoma sequence, similar to the adenoma of colonic polyps (Hsu 2014).

BP may be an incidental finding, as symptoms do not occur until the bile ducts are completely blocked by the tumor (Gu 2015).

Clinical symptoms may include:

- abdominal pain ((Alonso 2022)

- obstructive jaundice

- cholangitis (Hsu 2014).

Diagnosis is usually made predominantly by sonography, CT, or cholangiography-MRI (Alonso 2022). Intraluminal papillary tumors and dilated bile ducts are found (Lampichler 2019).

Misdiagnosis is not uncommon (Gu 2015) and sometimes the actual diagnosis can only be made intraoperatively (Gu 2015).

Abdominal sonography

Here, a hyperechoic mass with an irregular surface and uneven borders may be seen in the liver region (Gu 2015), as well as an enlarged cystic duct and choledochal duct (Deeg 2018).

Computed tomography

CT usually shows dilatation of the bile duct (Alonso 2022).

Mucin, which is formed by some papillomas, cannot be distinguished from bile on CT because both have the same signal intensity. Therefore, in the case of dilatation of the bile ducts, a neoplasia should be diagnosed even without direct evidence of tumor (Lampichler 2019).

Cholangiogram MRI

On cholangio- MRI, increases in size in particular can be observed (Alonso 2022).

Also in MRI, mucin, which is formed by some papillomas, cannot be distinguished from bile, as both have the same signal intensity. From this point of view, in case of dilatation of the bile ducts, a neoplasia should be placed even without direct tumor detection (Lampichler 2019).

Endoscopic retrograde cholangiopancreaticography (ERCP).

ERCP may reveal dilated bile ducts and filling defects (Hsu 2014).

FDG- PET / CT

This can be used to visualize the components of malignant transformation in particular ((Tu 2020).

There is sometimes a (slight) increase in:

- gamma-glutamyltransferase

- alkaline phosphatase (Gu 2015)

- Hyperbilirubinemia in icterus

Papillomas are divided into mucin-secreting or non-mucin-secreting papillomas (Hsu 2014).

Histologically, papillomas are classified as benign, but biliary papillomatosis represents a precancerous condition. Approximately 35% of affected individuals already have malignant transformation at the time of diagnosis (Gu 2015).

The differential diagnoses are complex for this relatively rare tumor (Gu 2015). These include, among others, cholangiocarcinoma (Gu 2015).

The rate of local recurrence is high, as is malignant transformation (Gu 2015).

Treatment consists of surgical resection (Hsu 2014).

A study by Wu (2021) showed that cholangioscopy with electrocoagulation is also a safe and effective alternative (Wu 2021).

If multiple organ invasion is already present, Xiao (2019) recommends adjuvant chemotherapy with e.g. Tegafur, Gimeracil and Oteracil- potassium capsules.

The 5-year survival rates are up to 81% after curative surgical resection (Gu 2015).

A longer follow-up period is required because both lobes of the liver are affected in approximately 1/3 of patients (Gu 2015).

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9. Wu C, Yang J F, Zhang Q, Liu W, Liao K, Hu B (2021) Successful cholangioscopic electrocoagulation for biliary papillomatosis: report covering six cases (with video). Gastroenterol Hepatol. 44 (8) 546 - 551
10. Xiao Y, Zhao J, Wu H, Xie K L, Wan Y, Xu X W, Zhang Y G (2019) Surgical treatment of malignant biliary papillomatosis invading adjacent organs: A case report. World J Clin Cases. 7 (2) 253 - 259