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WHO Classification of Tumours
Rhabdomyosarcoma, NOS
Liver and intrahepatic bile ducts


A rare aggressive malignant mesenchymal neoplasm arising from skeletal muscle. It usually occurs in children and young adults. Only a small percentage of tumors arise in the skeletal muscle of the extremities. The majority arise in other anatomical sites.

Hepatobiliary rhabdomyosarcoma (RMS)
RMS is a clinically and pathologically distinct tumour entity characterized by the development and growth of a usually embryonal- type rhabdomyosarcoma along the biliary tract in children

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Davis GL, Kissane JM, Ishak KG (1969)
Embryonal rhabdomyosarcoma (sarcoma botryoides) of the biliary tree. Report of five cases and a review of the literature.
Cancer 24: 333-42

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Lack EE, Perez-Atayde AR, Schuster SR (1981)
Botryoid rhabdomyosarcoma of the biliary tract.
Am J Surg Pathol 5: 643-52

. Although rare, this neoplasm is the most common tumour of the biliary tract in children, representing 1% of all paediatric rhabdomyosarcomas. The tumour occurs predominantly in infants, with a marked male preponderance, and about 2% of lesions are present at birth.

Clinical presentation is dominated by the sequelae of the tumour’s intrabiliary growth, i.e. mainly intermittent obstructive jaundice, but fever and nonspecific abdominal manifestations may also occur. Grossly, RMS forms soft and often transparent masses that grow into the biliary lumina, producing polypoid and grape-like masses (botryoid growth pattern). Definitive diagnosis requires immunohistochemistry, the neoplastic cells being immunoreactive for desmin, myogenin and MyoD.

For treatment, chemotherapy with or without surgery has been performed, leading to complete tumour regression in some patients and avoiding aggressive surgery, but treatment strategies and results are constantly changing owing to improved imaging procedures for staging and novel therapy regimes

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Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, Breneman J, Qualman SJ, Wiener E, Wharam M, Lobe T, Webber B, Maurer HM, Donaldson SS (2001)
Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease.
J Clin Oncol 19: 3091-102

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Huber J, Sovinz P, Freidl T, Jahnel J, Lackner H, Höllwarth M, Otte JB, Urban C (2008)
Long term survival in two children with rhabdomyosarcoma of the biliary tract.
Klin Padiatr 220: 378-9

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Martinez-F LA, Haase GM, Koep LJ, Akers DR (1982)
Rhabdomyosarcoma of the biliary tree: the case for aggressive surgery.
J Pediatr Surg 17: 508-11

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Pollono DG, Tomarchio S, Berghoff R, Drut R, Urrutia A, Cédola J (1998)
Rhabdomyosarcoma of extrahepatic biliary tree: initial treatment with chemotherapy and conservative surgery.
Med Pediatr Oncol 30: 290-3

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Ruymann FB, Raney RB, Crist WM, Lawrence W, Lindberg RD, Soule EH (1985)
Rhabdomyosarcoma of the biliary tree in childhood. A report from the Intergroup Rhabdomyosarcoma Study.
Cancer 56: 575-81

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Sanz N, de Mingo L, Flórez F, Rollán V (1997)
Rhabdomyosarcoma of the biliary tree.
Pediatr Surg Int 12: 200-1

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Spunt SL, Lobe TE, Pappo AS, Parham DM, Wharam MD, Arndt C, Anderson JR, Crist WM, Paidas C, Wiener E, Andrassy RJ, Schwartz CL (2000)
Aggressive surgery is unwarranted for biliary tract rhabdomyosarcoma.
J Pediatr Surg 35: 309-16

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Walterhouse D, Watson A (2007)
Optimal management strategies for rhabdomyosarcoma in children.
Paediatr Drugs 9: 391-400

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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010