8900/3 Rhabdomyosarcoma, NOS


Synonyms

Rhabdosarcoma

Definitions

Breast
ICD-O-3 topography code: C50
ICD10: C50
           

A malignant tumour composed of cells showing varying degrees of skeletal-muscle differentiation.






Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22
ICD10: C22
           

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 6
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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



7
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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
8
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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



9
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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



10
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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



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



12
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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



13
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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



14
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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



15
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Walterhouse D, Watson A (2007)
Optimal management strategies for rhabdomyosarcoma in children.
Paediatr Drugs 9: 391-400



16
 
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



.



Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72
ICD10: C70
           

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.



Esophagus
ICD-O-3 topography code: C15
ICD10: C15
           

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.

In the oesophagus, only a small number of well-documented rhabdomyosarcomas (of embryonal type) have been reported . These tumours have occurred in the distal oesophagus in older adults
17
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Vartio T, Nickels J, Höckerstedt K, Scheinin TM (1980)
Rhabdomyosarcoma of the oesophagus. Light and electron microscopic study of a rare tumor.
Virchows Arch A Pathol Anat Histol 386: 357-61



. Immunohistochemical demonstration of desmin and nuclear expression of myogenic regulatory proteins (MyoD1 or myogenin) is required for diagnosis.



Gallbladder and extrahepatic biliary tract
ICD-O-3 topography code: C23-C24.0
ICD10: C23-C24
           

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.

In young children, embryonal (botryoid) rhabdomyosarcoma occurs in the extrahepatic bile ducts and rarely in the gallbladder, where it can secondarily extend into the hepatic hilus and often manifests with biliary obstruction. Rhabdomyosarcoma forms a mass composed of multiple filiform, polypoid intraluminal protrusions involving the bile ducts
18
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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



19
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Al-Daraji WI, Makhlouf HR, Miettinen M, Montgomery EA, Goodman ZD, Marwaha JS, Fanburg-Smith JC (2009)
Primary gallbladder sarcoma: a clinicopathologic study of 15 cases, heterogeneous sarcomas with poor outcome, except pediatric botryoid rhabdomyosarcoma.
Am J Surg Pathol 33: 826-34



. Rhabdomyoblastic differentiation is variable, and coexpression of desmin and myogenic regulatory proteins (MYOD1, myogenin) is diagnostic. Prognosis is reasonably good with modern combined chemotherapy and surgery regimens; in one study, the estimated 5-year survival was 66% 20
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Nicol K, Savell V, Moore J, Teot L, Spunt SL, Qualman S, (2007)
Distinguishing undifferentiated embryonal sarcoma of the liver from biliary tract rhabdomyosarcoma: a Children's Oncology Group study.
Pediatr Dev Pathol 10: 89-97



21
 
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







Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33
ICD10: C13, C32, C33
           

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.



Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           

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.



Prostate gland
ICD-O-3 topography code: C61
ICD10: C61
           

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.



Urinary system: Renal pelvis, ureter, bladder, urethra
ICD-O-3 topography code: C65-C68
ICD10: C65-C68
           

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.



Thymus
ICD-O-3 topography code: C37
ICD10: C37
           

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.



Kidney
ICD-O-3 topography code: C64
ICD10: C64
           

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.