Extrahepatic bile ducts
Rhabdomyosarcoma of extrahepatic bile ducts

Author: Hanni Gulwani, M.D. (see Authors page)

Revised: 8 February 2018, last major update September 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Rhabdomyosarcoma of extrahepatic bile ducts

Cite this page: Gulwani, H. Rhabdomyosarcoma of extrahepatic bile ducts. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/gallbladderrhabdomyosarcoma.html. Accessed September 22nd, 2018.
Definition / general
  • < 1% of bile duct malignancies are sarcomas
  • Most common sarcoma is embryonal rhabdomyosarcoma
  • In children past infancy, #2 cause of obstructive jaundice (after choledochal cysts)
  • Usually 3 - 4 years old with fever, weight loss, jaundice
  • Usually embryonal or botryoid subtypes
  • 40% have metastases, usually to regional lymph nodes, distal metastases less common
  • Often extends into liver or gallbladder
  • Common bile duct (77%)
Case reports
  • Surgery, chemotherapy, radiation therapy may produce long term survival
Clinical images

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ERCP images

Gross description
  • Botryoid tumors have white tan, edematous, polypoid grape-like features
  • Tumor 3 - 14 cm
Microscopic (histologic) description
  • Embryonal tumors have condensed (cambium) layer of primitive spindle cells just below surface epithelium
  • Rhabdomyoblasts may be prominent with abundant eosinophilic cytoplasm
  • May have relatively acellular layer with myxoid stroma
Microscopic (histologic) images

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Some rhabdomyosarcoma cells have eosinophilic cytoplasm suggesting skeletal muscle differentiation but most are very undifferentiated in myxoid stroma; tumor cells are strongly myogenenin+

Positive stains
Electron microscopy description
  • Thick and thin filaments