II. Epidemiology

  1. Most common soft tissue Sarcoma in children
  2. Responsible for up to 8% of Childhood Cancer

III. Distribution

  1. May be located anywhere
  2. Head and neck: 40%
  3. Genitourinary tract: 20%
  4. Extremities: 20%
  5. Trunk: 10%

IV. Risk factors

V. Presentation: Local swelling and soft tissue displacement

  1. Usually painless
  2. Compression Neuropathy (e.g. Cranial Nerve palsy)
  3. Central nervous system involvement

VI. Histologic Types

  1. Embroyonal (60%)
    1. Intermediate prognosis
  2. Alveolar (15%)
    1. Named for alveoli-like clefts
    2. Usually involves trunk or extremities
    3. Worst prognosis of Rhabdomyosarcomas
  3. Botryoid (6%)
    1. Forms grap-like clusters
    2. Tumors of nasopharynx and genitourinary tract
  4. Pleomorphic (1% in children, usually seen in adults)

VII. Management

  1. Complete local excision if possible
    1. Directed by site and adjacent structures
    2. More limited excision at orbit, genitourinary tract
    3. Regional lymph node sampling is also performed
  2. Adjunctive management
    1. Chemotherapy
    2. Radiation
    3. Further surgical excision

VIII. References

  1. Crist in Behrman (2000) Nelson Pediatrics, p. 1557-8
  2. Andrassy (2002) Am J Surg 184:484-91 [PubMed]

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Ontology: Rhabdomyosarcoma (C0035412)

Definition (NCI_CDISC) A malignant mesenchymal neoplasm arising from skeletal muscle.
Definition (NCI) 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.
Definition (NCI_NCI-GLOSS) Cancer that forms in the soft tissues in a type of muscle called striated muscle. Rhabdomyosarcoma can occur anywhere in the body.
Definition (MSH) A malignant solid tumor arising from mesenchymal tissues which normally differentiate to form striated muscle. It can occur in a wide variety of sites. It is divided into four distinct types: pleomorphic, predominantly in male adults; alveolar (RHABDOMYOSARCOMA, ALVEOLAR), mainly in adolescents and young adults; embryonal (RHABDOMYOSARCOMA, EMBRYONAL), predominantly in infants and children; and botryoidal, also in young children. It is one of the most frequently occurring soft tissue sarcomas and the most common in children under 15. (From Dorland, 27th ed; Holland et al., Cancer Medicine, 3d ed, p2186; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, pp1647-9)
Definition (CSP) highly malignant tumor of striated muscle derived from primitive mesenchymal cells and exhibiting differentiation along rhabdomyoblastic lines.
Concepts Neoplastic Process (T191)
MSH D012208
ICD10 M8900/3
SnomedCT 30924005, 134166003, 302847003
English Rhabdomyosarcomas, [M]Rhabdomyosarcoma NOS, Rhabdomyosarcoma NOS, Rhabdomyosarcoma, no subtype, rhabdomyosarcoma (diagnosis), malignant neoplasm myosarcoma rhabdomyosarcoma, rhabdomyosarcoma, Rhabdomyosarcoma [Disease/Finding], rhabdomyosarcomas, [M]Rhabdomyosarcoma NOS (morphologic abnormality), RHABDOMYOSARCOMA, MALIGNANT, Rhabdomyosarcoma, NOS, Rhabdosarcoma, Rhabdomyosarcoma (disorder), Rhabdomyosarcoma, no subtype (morphologic abnormality), Rhabdomyosarcoma, rhabdomyoblastoma, rhabdosarcoma
Spanish morfología: rabdomiosarcoma, no clasificado como subtipo, Rabdomiosarcoma NEOM, Rabdosarcoma, [M] rabdomiosarcoma SAI (anomalía morfológica), [M] rabdomiosarcoma SAI, morfología: rabdomiosarcoma, no clasificado como subtipo (anomalía morfológica), rabdomiosarcoma (trastorno), rabdomiosarcoma, rabdosarcoma, Rabdomiosarcomas, Rabdomiosarcoma
Italian Rabdomiosarcomi, Rabdosarcoma, Rabdomiosarcoma NAS, Rabdomiosarcoma
Dutch rabdomyosarcoom NAO, rabdosarcoom, rabdomyosarcomata, rabdomyosarcoom, Rabdomyosarcoom, Sarcoom, rabdomyo-
French Rhabdomyosarcome SAI, Rhabdosarcome, Rhabdomyosarcomes, Rhabdomyosarcome
German Rhabdomyosarkom NNB, Rhabdomyosarkome, Rhabdomyosarkom
Portuguese Rabdomiossarcoma NE, Rabdossarcoma, Rabdomiossarcomas, Rabdomiossarcoma
Japanese 横紋筋肉腫NOS, 横紋肉腫, 横紋筋肉腫, オウモンキンニクシュ, オウモンニクシュ, オウモンキンニクシュNOS
Swedish Rabdomyosarkom
Czech rabdomyosarkom, rhabdomyosarkom, Rabdomyosarkom, Rabdomyosarkom NOS, Rabdomyosarkomy, Rabdosarkom
Finnish Rabdomyosarkooma
Russian RABDOMIOSARKOMA, РАБДОМИОСАРКОМА
Polish Mięśniakomięsak prążkowanokomórkowy, Mięsak mięśni prążkowanych
Hungarian Rhabdomyosarcoma, Rhabdomyosarcomak, Rhabdomyosarcoma k.m.n., Harántcsíkolt izom rosszindulatú daganata
Norwegian Rabdomyosarkom