Extrahepatic bile ducts
Rhabdomyosarcoma of extrahepatic bile ducts

Topic Completed: 1 September 2012

Revised: 13 September 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Rhabdomyosarcoma of extrahepatic bile ducts

Hanni Gulwani, M.B.B.S.
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Cite this page: Gulwani H. Rhabdomyosarcoma of extrahepatic bile ducts. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/gallbladderrhabdomyosarcoma.html. Accessed February 18th, 2020.
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
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