Intrahepatic Carcinosarcoma With Cholangiocarcinoma Elements and Prominent Bile Duct Spread

Int J Surg Pathol. 2019 Dec;27(8):900-906. doi: 10.1177/1066896919855766. Epub 2019 Jun 17.

Abstract

Carcinosarcomas with elements of cholangiocarcinoma and sarcoma are rare and have a poor prognosis. The spreading pattern and radiological findings of these lesions remain unclear. A 74-year-old man presented with a high γ-glutamyl transferase level. Magnetic resonance imaging revealed dilation of the right intrahepatic and common bile ducts, consistent with an intraductal papillary neoplasm of the bile duct (IPNB), and diffusion-weighted imaging (DWI) indicated an area of high signal intensity in the intrahepatic bile duct. Bile duct biopsy yielded a small amount of atypical spindle cells, and the patient underwent a right hepatectomy. Microscopically, the tumor contained cholangiocarcinoma and sarcomatous components, including osteosarcoma and leiomyosarcoma, leading to a diagnosis of intrahepatic carcinosarcoma. The tumor spread primarily through the intrahepatic bile duct. An accurate radiological diagnosis of carcinosarcoma was challenging, given the apparent similarities with IPNB. The findings from DWI and pathology of a bile duct biopsy may assist with preoperative diagnosis.

Keywords: carcinosarcoma; cholangiocarcinoma; endoscopic retrograde cholangiopancreatography; intraductal papillary neoplasm of the bile duct; intrahepatic; leiomyosarcoma; liver; osteosarcoma; primary hepatic carcinosarcoma; sarcomatous carcinoma.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Asymptomatic Diseases / therapy
  • Bile Duct Neoplasms / blood
  • Bile Duct Neoplasms / diagnosis*
  • Bile Duct Neoplasms / pathology
  • Bile Duct Neoplasms / therapy
  • Bile Ducts, Intrahepatic / diagnostic imaging
  • Bile Ducts, Intrahepatic / pathology*
  • Bile Ducts, Intrahepatic / surgery
  • Biopsy
  • Carcinosarcoma / blood
  • Carcinosarcoma / diagnosis*
  • Carcinosarcoma / pathology
  • Carcinosarcoma / therapy
  • Cholangiocarcinoma / blood
  • Cholangiocarcinoma / diagnosis*
  • Cholangiocarcinoma / pathology
  • Cholangiocarcinoma / therapy
  • Cholangiopancreatography, Endoscopic Retrograde
  • Embolization, Therapeutic
  • Hepatectomy
  • Humans
  • Liver Function Tests
  • Magnetic Resonance Imaging
  • Male
  • Portal Vein
  • Ultrasonography
  • gamma-Glutamyltransferase / blood

Substances

  • gamma-Glutamyltransferase