Abstract

Fewer than 90 cases of granular cell tumour (GCT) of the biliary tract have been reported, including only five cases of multiple GCTs. We present the unusual case of a 40-year-old woman with multifocal GCTs affecting the intrahepatic biliary tree, which were initially suspected to be hepatic multiple metastases from a malignancy of unknown origin. The surgical specimen consisted of a hepatic segment in which five whitish nodular lesions were observed. On microscopic examination, nodular lesions were found in the portal tracts; these were composed of large polygonal cells with abundant highly granular cytoplasm. The nuclei were small and centrally located. The tumour cells tested diffusely positive for CD68-PGM1, S100 protein and α-inhibin, so a diagnosis of multifocal GCT of the biliary tree was made. Three years later, the patient is still alive and a MRI has shown no changes.