Multifocal granular cell tumour of the biliary tree

BMJ Case Rep. 2018 Nov 28;11(1):e226352. doi: 10.1136/bcr-2018-226352.

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.

Keywords: gastrointestinal surgery; pathology; radiology.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aftercare
  • Antigens, CD / metabolism
  • Antigens, Differentiation, Myelomonocytic / metabolism
  • Biliary Tract / diagnostic imaging*
  • Biliary Tract / pathology*
  • Biliary Tract / ultrastructure
  • Female
  • Granular Cell Tumor / diagnostic imaging*
  • Granular Cell Tumor / pathology*
  • Granular Cell Tumor / ultrastructure
  • Humans
  • Inhibins / metabolism
  • Magnetic Resonance Imaging / methods
  • Positron Emission Tomography Computed Tomography / methods
  • S100 Proteins / metabolism

Substances

  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • CD68 antigen, human
  • S100 Proteins
  • inhibin-alpha subunit
  • Inhibins