Rhabdomyosarcoma epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Overview

Epidemiology and Demographics

  • Rhabdomyosarcoma is considered as a most common soft tissue cancers among children and adolescents and it is the third most common extracranial solid tumors during childhood which comes after neuroblastomaand wilms tumor.[1][2]
  • Rhabdomyosarcoma is responsible for 50% of soft tissue tumors during childhood and 5% of all pediatrics cancers.[3]
  • Almost 350 new cases of rhabdomyosarcoma are diagnosed annually.[1]

Incidence

  • The incidence of rhabdomyosarcomais about 0.43 per 100,000 annually among children,adolescents, and young adults under 20 years old.[1]
  • The incidence may depend on the histologic subtype of rhabdomyosarcoma:[4]
    • Embryonal rhabdomyosarcoma: Embryonal rhabdomyosarcoma peaks in 0 to 4 year age group at approximately 0.4 cases per 100,000 children, with a lower rate in adolescents, approximately 0.15 cases per 100,000 adolescents.
    • Alveolar rhabdomyosarcoma: The incidence of alveolar rhabdomyosarcoma does not vary by gender and is constant from ages 0 to 19 years at approximately 0.1 case per 100,000 children and adolescents.

Case-fatality rate/Mortality rate

  • In patients with localized disease, the estimated 5-year survival rate is greater than 80% after using surgery,radiation therapy, and chemotherapy for treatment.[5]
  • In patients with metastatic lesions, the estimated 5-year event-free survival is less than 30%.[6]
  • The 3-year event-free survival rate for patients with metastatic lesions without high risk factors (bone marrow involvement, unfavorable site, more than 3 sites, and age younger than 1 year or older than 10 years)is approximately 50%.
  • The 3-year event-free survival rate for patients with metastatic lesions with 3 high risk factors is about 12% and with 4 high risk factors is 5%.
  • The 5-year survival rate is 86% for orbital site, 80% for genitourinary site, 50% for extremities, 52% for retroperitoneum and trunk.[7]
  • The 5-year survival rate is 77% for patients aged 1-4 years, 47% for infants and 48% for adolescents.

Age

  • Approximately two-third of all cases happen under 6 years old.[8]
  • The peak incidence of rhabdomyosarcoma is between 2-6 years and 10-18 years.
  • Embryonal rhabdomyosarcoma is usually happen in younger ages especially develops in head/neck sites and genitourinary locations.
  • Alveolar rhabdomyosarcoma is usually happen in older patients (adolescents) especially develops in trunk and extremities sites.

Race

  • The incidence of rhabdomyosarcoma is higher among black patients than white patients particularly between 15-19 years.[9]

Gender

Region

  • The incidence of rhabdomyosarcoma is higher among Caucasian in comparison to Asian population.[10]

References

  1. 1.0 1.1 1.2 Dasgupta R, Fuchs J, Rodeberg D (2016). "Rhabdomyosarcoma". Semin Pediatr Surg. 25 (5): 276–283. doi:10.1053/j.sempedsurg.2016.09.011. PMID 27955730.
  2. Arndt CA, Crist WM (1999). "Common musculoskeletal tumors of childhood and adolescence". N Engl J Med. 341 (5): 342–52. doi:10.1056/NEJM199907293410507. PMID 10423470.
  3. Dasgupta R, Rodeberg DA (2012). "Update on rhabdomyosarcoma". Semin Pediatr Surg. 21 (1): 68–78. doi:10.1053/j.sempedsurg.2011.10.007. PMID 22248972.
  4. Ognjanovic S, Linabery AM, Charbonneau B, Ross JA (2009). "Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975-2005". Cancer. 115 (18): 4218–26. doi:10.1002/cncr.24465. PMC 2953716. PMID 19536876.
  5. Punyko JA, Mertens AC, Baker KS, Ness KK, Robison LL, Gurney JG (2005). "Long-term survival probabilities for childhood rhabdomyosarcoma. A population-based evaluation". Cancer. 103 (7): 1475–83. doi:10.1002/cncr.20929. PMID 15712283.
  6. Oberlin O, Rey A, Lyden E, Bisogno G, Stevens MC, Meyer WH; et al. (2008). "Prognostic factors in metastatic rhabdomyosarcomas: results of a pooled analysis from United States and European cooperative groups". J Clin Oncol. 26 (14): 2384–9. doi:10.1200/JCO.2007.14.7207. PMC 4558625. PMID 18467730.
  7. Mazzoleni S, Bisogno G, Garaventa A, Cecchetto G, Ferrari A, Sotti G; et al. (2005). "Outcomes and prognostic factors after recurrence in children and adolescents with nonmetastatic rhabdomyosarcoma". Cancer. 104 (1): 183–90. doi:10.1002/cncr.21138. PMID 15895378.
  8. Miller, Robert W.; L Young, John; Novakovic, Biljana (1995). "Childhood cancer". Cancer. 75 (S1): 395–405. doi:10.1002/1097-0142(19950101)75:1+<395::AID-CNCR2820751321>3.0.CO;2-W. ISSN 0008-543X.
  9. Paulino AC, Okcu MF (2008). "Rhabdomyosarcoma". Curr Probl Cancer. 32 (1): 7–34. doi:10.1016/j.currproblcancer.2007.11.001. PMID 18206520.
  10. Stiller CA, McKinney PA, Bunch KJ, Bailey CC, Lewis IJ (1991). "Childhood cancer and ethnic group in Britain: a United Kingdom children's Cancer Study Group (UKCCSG) study". Br J Cancer. 64 (3): 543–8. PMC 1977662. PMID 1654982.

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