Author(s): Almeida e Sousa, M ; Carvalho, A ; Mega, R ; Bilhim, T
Date: 2022
Persistent ID: http://hdl.handle.net/10400.17/4513
Origin: Repositório do Centro Hospitalar de Lisboa Central, EPE
Subject(s): Bile Duct Neoplasms; Hepatectomy;; Hepatic Duct, Common; Klatskin Tumour; Liver Function Tests; IMA; ANPAT
Inflammatory myofibroblastic tumour is a rare entity of indeterminate biological potential with a reduced tendency for recurrence and metastasis. Although it can arise from multiple organs, the bile duct is a very rare site of origin. We report the case of a 75-year-old asymptomatic male with elevated gamma-glutamyl transferase [1575 U/L (12 - 64 U/L)] and alkaline phosphatase [271 U/L (40 - 150 U/L)]. Computed tomography showed a 17 mm hypervascular lesion in the confluence of the right and left hepatic ducts, with bile duct ectasia and right liver lobe atrophy. The patient was initially managed as having a Klatskin tumour and underwent right hepatectomy. Histology showed a spindle cell proliferation with an inflammatory infiltrate of lymphocytes, plasma cells and collagen-rich stroma, consistent with an inflammatory myofibroblastic tumour. He was discharged 30 days after admission, and nine months later remains asymptomatic. His liver function tests have normalized and follow-up tests are unremarkable.
