BACKGROUND: Rhabdomyosarcoma survivors have an increased risk of developing second malignant neoplasms (SMN); this risk is traditionally attributed to the effects of multidisciplinary management required for cure. However, the impact of constitutional predisposition has not been properly analyzed. METHODS: We analyzed the risk of SMN among 1,151 children diagnosed with rhabdomyosarcoma and reported to the Surveillance, Epidemiology, and End Results registries (SEER-9) from 1973 to 2010. Standardized incidence ratios (SIR) and corresponding 95% confidence intervals (CI) were calculated using SEERStat 8.1.2. RESULTS: Children with pleomorphic and embryonal rhabdomyosarcoma had an increased risk of developing a SMN (SIR = 15.77, 95%CI 1.91-56.96 and SIR = 5.6, 95%CI 3.32-8.85, respectively). The risk was age-dependent; the highest was among children <2 years (SIR = 13.38, 95%CI 4.34-31.22) and the lowest was in children >10 years (SIR = 3.35, 95%CI 1.53-6.35). The risk for the youngest patients was higher for those with embryonal rhabdomyosarcoma (SIR = 14.72, 95%CI 4.01-37.70) compared to other histiotypes. Additionally, the risk of SMN was independent of the use of radiation to the primary (SIR = 6.50, 95%CI 3.97-10.03 and SIR = 4.57, 95%CI 2.09-8.68, for children receiving and not receiving radiation, respectively). The pattern of SMN observed was consistent with the Li-Fraumeni spectrum. CONCLUSIONS: Children with rhabdomyosarcoma are at high risk of developing SMN. This risk is higher for a subgroup of young children with pleomorphic and embryonal histologies, and is independent of the use of radiation. This suggests that a subgroup of children with pleomorphic and embryonal rhabdomyosarcoma may have a constitutional cancer predisposition.
BACKGROUND:Rhabdomyosarcoma survivors have an increased risk of developing second malignant neoplasms (SMN); this risk is traditionally attributed to the effects of multidisciplinary management required for cure. However, the impact of constitutional predisposition has not been properly analyzed. METHODS: We analyzed the risk of SMN among 1,151 children diagnosed with rhabdomyosarcoma and reported to the Surveillance, Epidemiology, and End Results registries (SEER-9) from 1973 to 2010. Standardized incidence ratios (SIR) and corresponding 95% confidence intervals (CI) were calculated using SEERStat 8.1.2. RESULTS:Children with pleomorphic and embryonal rhabdomyosarcoma had an increased risk of developing a SMN (SIR = 15.77, 95%CI 1.91-56.96 and SIR = 5.6, 95%CI 3.32-8.85, respectively). The risk was age-dependent; the highest was among children <2 years (SIR = 13.38, 95%CI 4.34-31.22) and the lowest was in children >10 years (SIR = 3.35, 95%CI 1.53-6.35). The risk for the youngest patients was higher for those with embryonal rhabdomyosarcoma (SIR = 14.72, 95%CI 4.01-37.70) compared to other histiotypes. Additionally, the risk of SMN was independent of the use of radiation to the primary (SIR = 6.50, 95%CI 3.97-10.03 and SIR = 4.57, 95%CI 2.09-8.68, for children receiving and not receiving radiation, respectively). The pattern of SMN observed was consistent with the Li-Fraumeni spectrum. CONCLUSIONS:Children with rhabdomyosarcoma are at high risk of developing SMN. This risk is higher for a subgroup of young children with pleomorphic and embryonal histologies, and is independent of the use of radiation. This suggests that a subgroup of children with pleomorphic and embryonal rhabdomyosarcoma may have a constitutional cancer predisposition.
Authors: Meerim Park; Jun Ah Lee; Hye Young Jin; Joo-Young Kim; Jong Woong Park; June Hyuk Kim; Hyun Guy Kang; Seog Yun Park; Eun Young Park; Hyeon Jin Park; Byung Kiu Park Journal: J Cancer Res Clin Oncol Date: 2022-07-23 Impact factor: 4.322
Authors: Amy L Sherborne; Vincent Lavergne; Katharine Yu; Leah Lee; Philip R Davidson; Tali Mazor; Ivan V Smirnoff; Andrew E Horvai; Mignon Loh; Steven G DuBois; Robert E Goldsby; Joseph P Neglia; Sue Hammond; Leslie L Robison; Rosanna Wustrack; Joseph F Costello; Alice O Nakamura; Kevin M Shannon; Smita Bhatia; Jean L Nakamura Journal: Clin Cancer Res Date: 2016-09-28 Impact factor: 12.531
Authors: Brandon J Diessner; Brenda J Weigel; Paari Murugan; Lin Zhang; Jenny N Poynter; Logan G Spector Journal: Cancer Epidemiol Biomarkers Prev Date: 2020-09-14 Impact factor: 4.254
Authors: M D Mathias; M V Ortiz; H Magnan; S R Ambati; E K Slotkin; A J Chou; M F Walsh; K Offit; C Moskowitz; A Kentsis; L H Wexler Journal: Biomark Res Date: 2017-02-08