Liver and intrahepatic bile ducts - tumor
Other malignancies

Topic Completed: 1 February 2012

Revised: 28 January 2019, last major update February 2012

Copyright: (c) 2002-2017,, Inc.

PubMed Search: Rhabdomyosarcoma[TI] liver[TIAB]

Deepali Jain, M.D.
Page views in 2019: 272
Page views in 2020 to date: 111
Cite this page: Jain D. Rhabdomyosarcoma. website. Accessed April 6th, 2020.
Definition / general
  • Rare but most common biliary tract tumor in children (1% of pediatric rhabdomyosarcoma), usually embronal subtype, usually infant males (IARC: 8900 / 3 Rhabdomyosarcoma, NOS [Accessed 5 December 2017])
  • May have secondary spread into liver
  • Very rare in adults (< 15 cases reported to date), females > males
  • Present with obstructive jaundice
  • May be associated with undifferentiated sarcoma
  • Poor prognosis
  • Surgical resection, chemoradiation therapy
Gross description
  • Polypoid myxoid mass extending into bile duct
Microscopic (histologic) description
  • Usually botryoid type embryonal rhabdomyosarcoma with soft polypoid masses covered by biliary type epithelium protruding into ductal lumen
  • Cells are small, hyperchromatic with rare cross striations in eosinophilic cytoplasm
  • Cambium layer (densely packed cells beneath biliary epithelium) is present, occasional tumor giant cells with cross striations
  • Myxoid stroma under cambium layer; no hyaline globules
Microscopic (histologic) images

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Paratesticular embyronal rhabdomyosarcoma

Electron microscopy description
  • May show thin and thick filaments
Differential diagnosis
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