Hematology and Oncology Book

Anemia

Sarcomas

  • Rhabdomyosarcoma

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Rhabdomyosarcoma

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  1. See Also
    1. Sarcoma
  2. Epidemiology
    1. Most common soft tissue Sarcoma in children
    2. Responsible for up to 8% of childhood cancer
  3. Distribution
    1. May be located anywhere
    2. Head and neck: 40%
    3. Genitourinary tract: 20%
    4. Extremities: 20%
    5. Trunk: 10%
  4. Risk factors
    1. Neurofibromatosis
  5. Presentation: Local swelling and soft tissue displacement
    1. Usually painless
    2. Compression Neuropathy (e.g. Cranial Nerve palsy)
    3. Central nervous system involvement
  6. 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)
  7. 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
  8. References
    1. Crist in Behrman (2000) Nelson Pediatrics, p. 1557-8
    2. Andrassy (2002) Am J Surg 184:484

Rhabdomyosarcoma (C0035412)

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.
Definition (NCI)A malignant tumor of muscle tissue.
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.
ConceptsNeoplastic Process (T191)
MSHD012208
Englishrhabdomyoblastoma, Rhabdomyosarcoma, Rhabdomyosarcomas, Rhabdosarcoma
Spanishrabdomiosarcoma, rabdosarcoma
Parent Conceptssarcoma (C1261473), Myomatous neoplasm (C0282606), Myosarcoma (C0027095), Infrequent Neoplasm (C1334185), Malignant Skeletal Muscle Neoplasm (C1334619), Sarcoma NCI Grade 3 (C1335918), Rhabdomyomatous neoplasm (C0476135), Rhabdomyosarcoma - category (C1276181), Primary malignant neoplasm (C1306459)
SourcesAOD, COSTAR, CSP, DXP, LCH, MEDLINEPLUS, MSH, MTH, NCI, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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