Rhabdomyosarcoma epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Shadan Mehraban, M.D.[2]
Overview
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 neuroblastoma and wilms tumor. Rhabdomyosarcoma is responsible for 50% of soft tissue tumors during childhood and 5% of all pediatrics cancers. Approximately two-third of all cases happen under 6 years old.The incidence of rhabdomyosarcoma is about 0.43 per 100,000 annually among children, adolescents, and young adults under 20 years old. In patients with localized disease, the estimated 5-year survival rate is greater than 80% after using surgery, radiation therapy, and chemotherapy for treatment and in patients with metastatic lesions, the estimated 5-year event-free survival is less than 30%. The incidence of rhabdomyosarcoma is a little more predominant in males than females.
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 neuroblastoma and 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 rhabdomyosarcoma is 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
- The incidence of rhabdomyosarcoma is a little more predominant in males than females (about 1.3:1-1.5:1).[9]
Region
- The incidence of rhabdomyosarcoma is higher among Caucasian population in comparison to Asian population.[10]
References
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 9.0 9.1 Paulino AC, Okcu MF (2008). "Rhabdomyosarcoma". Curr Probl Cancer. 32 (1): 7–34. doi:10.1016/j.currproblcancer.2007.11.001. PMID 18206520.
- ↑ 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.