Liver and intrahepatic bile ducts - tumor
Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.
● 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
● Surgical resection, chemoradiation therapy
● Polypoid myxoid mass extending into bile duct
● 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
Parataesticular embyronal rhabdomyosarcoma
● Desmin, muscle specific actin, myoglobin and MyoD1
Electron microscopy description
● May show thin and thick filaments
● Malignant rhabdoid tumor: negative for MyoD1, myoglobin
End of Liver and intrahepatic bile ducts - tumor > Other malignancies > Rhabdomyosarcoma
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