• Matilde Almeida E Sousa, Ana Carvalho, Raquel Mega, and Tiago Bilhim.
• Serviço de Radiologia. Centro Hospitalar Universitário Lisboa Central. Lisboa. Departamento de Anatomia e Imagiologia Clínicas. NOVA Medical School. Lisboa. Portugal.
• Acta Medica Port. 2022 Feb 7; 35 (10).

AbstractInflammatory 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.

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