Bone and Soft-Tissue Sarcoma Risk in Long-Term Survivors of Hereditary Retinoblastoma Treated With Radiation
Bone and Soft-Tissue Sarcoma Risk in Long-Term Survivors of Hereditary Retinoblastoma Treated With Radiation
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DOI：
10.1200/jco.19.01096
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发表时间：
2019-12-10
影响因子：
45.3
通讯作者：
Morton, Lindsay M.
中科院分区：
文献类型：
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作者：
Kleinerman, Ruth A.;Schonfeld, Sara J.;Morton, Lindsay M.
目的遗传性视网膜母细胞瘤的存活者有很好的存活率，但随后发生骨和软组织肉瘤的风险显著增加，特别是在放射治疗后。对肉瘤风险模式的全面调查将有助于临床对幸存者的监测。方法与方法在1914-2006年间首次诊断为遗传性视网膜母细胞瘤的952名受辐射的幸存者中，我们用标准化发病率(SIRS)和累积发病率分析来量化肉瘤风险。结果105例骨肉瘤和124例软组织肉瘤中，超过一半发生在头颈部(骨，53.3%；软组织，51.6%)，四分之一(骨，29.5%；软组织，25.0%)，约五分之一(骨，17.1%；软组织，23.4%)。我们注意到，与普通人群相比，头部和颈部相对于身体和四肢肿瘤的风险要高得多(SIR，2213；95%CI，1,671至2873v SIR，169；95%CI，115至239)和软组织肉瘤(SIR，542；95%CI，418至692v SIR，45.7；95%CI，31.1至64.9)。头颈部骨肉瘤和软组织肉瘤从儿童早期开始诊断，一直持续到成年，60年累计发病率分别为6.8%(95%可信区间，5.0%~8.7%)和9.3%(95%可信区间，7.0%~11.7%)。相反，身体和四肢骨肉瘤发病率在青春期后趋于平缓(3.5%；95%可信区间，2.3%~4.8%)，而身体和肢体软组织肉瘤发病率在30岁之前很少见(60年累积发病率，6.6%；95%可信区间，4.1%~9.2%)，尤其是女性(9.4%；95%可信区间，5.1%至13.8%)。结论骨和软组织肉瘤发病率显著升高的年龄、部位和性别不同，突出了放射治疗和遗传易感性的重要作用。这些数据为制定基于风险的筛查方案提供了指导，该方案重点关注按年龄、位置和性别划分的最高肉瘤风险。(C)2019年，由美国临床肿瘤学会主办。
PURPOSE Survivors of hereditary retinoblastoma have excellent survival but substantially increased risks of subsequent bone and soft-tissue sarcomas, particularly after radiotherapy. Comprehensive investigation of sarcoma risk patterns would inform clinical surveillance for survivors.PATIENTS AND METHODS In a cohort of 952 irradiated survivors of hereditary retinoblastoma who were originally diagnosed during 1914 to 2006, we quantified sarcoma risk with standardized incidence ratios (SIRs) and cumulative incidence analyses. We conducted analyses separately for bone and soft-tissue sarcomas occurring in the head and neck (in/near the radiotherapy field) versus body and extremities (out of field).RESULTS Of 105 bone and 124 soft-tissue sarcomas, more than one half occurred in the head and neck (bone, 53.3%; soft tissue, 51.6%), one quarter in the body and extremities (bone, 29.5%; soft tissue, 25.0%), and approximately one fifth in unknown/unspecified locations (bone, 17.1%; soft tissue, 23.4%). We noted substantially higher risks compared with the general population for head and neck versus body and extremity tumors for both bone (SIR, 2,213; 95% CI, 1,671 to 2,873 v SIR, 169; 95% CI, 115 to 239) and soft-tissue sarcomas (SIR, 542; 95% CI, 418 to 692 v SIR, 45.7; 95% CI, 31.1 to 64.9). Head and neck bone and soft-tissue sarcomas were diagnosed beginning in early childhood and continued well into adulthood, reaching a 60-year cumulative incidence of 6.8% (95% CI, 5.0% to 8.7%) and 9.3% (95% CI, 7.0% to 11.7%), respectively. In contrast, body and extremity bone sarcoma incidence flattened after adolescence (3.5%; 95% CI, 2.3% to 4.8%), whereas body and extremity soft-tissue sarcoma incidence was rare until age 30, when incidence rose steeply (60-year cumulative incidence, 6.6%; 95% CI, 4.1% to 9.2%), particularly for females (9.4%; 95% CI, 5.1% to 13.8%).CONCLUSION Strikingly elevated bone and soft-tissue sarcoma risks differ by age, location, and sex, highlighting important contributions of both radiotherapy and genetic susceptibility. These data provide guidance for the development of a risk-based screening protocol that focuses on the highest sarcoma risks by age, location, and sex. (C) 2019 by American Society of Clinical Oncology.