Liver and intrahepatic bile ducts - tumor

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Liver and intrahepatic bile ducts - tumor

Other malignancies

Rhabdomyosarcoma

Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.

General
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● Rare, but most common biliary tract tumor in children (1% of pediatric rhabdomyosarcoma), usually embronal subtype, usually infant males (WHO)
● 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

Treatment
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● Surgical resection, chemoradiation therapy

Gross description
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● Polypoid myxoid mass extending into bile duct

Micro description
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● Usually botyroid-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

Micro images
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Various images

Parataesticular embyronal rhabdomyosarcoma

Positive stains
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● Desmin, muscle specific actin, myoglobin and MyoD1

Electron microscopy description
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● May show thin and thick filaments

Differential diagnosis
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● Malignant rhabdoid tumor: negative for MyoD1, myoglobin

End of Liver and intrahepatic bile ducts - tumor > Other malignancies > Rhabdomyosarcoma

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