Pediatrics 1, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
Pediatrics 5, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
Cancer. 2022 May 1;128(9):1787-1800. doi: 10.1002/cncr.34110. Epub 2022 Feb 23.
Increased survival in young sarcoma patients comes along with a higher incidence of second malignant neoplasms (SMNs). The incidence, latency, histiotype, and outcome of these patients were analyzed because this information is essential to design evidence-based long-term follow-up care programs for young sarcoma survivors.
Patients entered on clinical trials or registered in registries with a primary sarcoma in 1 of the cooperative sarcoma study groups in the framework of the Society for Pediatric Oncology and Hematology (GPOH) were screened for SMNs. Descriptive analysis, the Kaplan-Meier method, the Gray model, the Fine-Gray model, and the Cox regression model were used for the statistical analyses.
A total of 159 out of 7079 (2.2%) patients were registered with a SMN. Among them, 104 solid SMNs (65%) and 56 hematologic SMNs (35%) occurred. Median latency from first diagnosis of sarcoma to the diagnosis of SMN was 6.8 years (range, 0-26.7 years). Cumulative incidence of SMN was 8.8% after 30 years. Five-year-survival was 67.1% (95% confidence interval [CI], 66.0-68.2) for the 7079 patients and it was 45.1% (95% CI, 36.2-53.6) after the diagnosis of a SMN (subcohort of n = 159 patients).
There is a remarkable high cumulative incidence of SMNs after bone and soft tissue sarcomas in children, adolescents, and young adults. Therefore, effective transition as well as risk adapted long-term follow-up care programs should be developed and offered to young sarcoma survivors.
Bone sarcomas and soft tissue tumors are rare tumors in children, adolescents, and young adults. The treatment varies, but may comprise chemotherapy, surgery, and/or radiotherapy. Developing a subsequent malignant tumor is a long-term risk for the patients. To better characterize this risk, we analyzed the data of 7079 patients (up to 21 years old) with bone sarcomas or soft tissue tumors. Our findings provide a basis to counsel young sarcoma survivors on their individual risk of subsequent malignant tumors. Moreover, these data can help to establish recommendations for aftercare in young sarcoma survivors.
年轻肉瘤患者的生存率提高伴随着第二恶性肿瘤（SMN）发病率的上升。分析这些患者的发病率、潜伏期、组织类型和结局，因为这些信息对于设计基于证据的年轻肉瘤幸存者长期随访护理计划至关重要。
在儿科肿瘤学和血液学协会（GPOH）合作肉瘤研究组的临床试验或登记处登记有原发性肉瘤的患者中，筛选出 SMN。采用描述性分析、Kaplan-Meier 法、Gray 模型、Fine-Gray 模型和 Cox 回归模型进行统计学分析。
在 7079 名患者中，共有 159 名（2.2%）患者登记为 SMN。其中，104 例为实体 SMN（65%），56 例为血液学 SMN（35%）。从首次诊断肉瘤到诊断 SMN 的中位潜伏期为 6.8 年（范围 0-26.7 年）。30 年后 SMN 的累积发病率为 8.8%。7079 名患者的 5 年生存率为 67.1%（95%置信区间[CI]：66.0-68.2），而在诊断为 SMN 后（n=159 名患者的亚组）的 5 年生存率为 45.1%（95%CI：36.2-53.6）。
儿童、青少年和年轻成人的骨和软组织肉瘤后，SMN 的累积发病率明显较高。因此，应制定并提供有效的过渡和风险适应的长期随访护理计划，以满足年轻肉瘤幸存者的需求。
骨肉瘤和软组织肿瘤是儿童、青少年和年轻成人中罕见的肿瘤。治疗方法各不相同，但可能包括化疗、手术和/或放疗。对于患者来说，发展为继发恶性肿瘤是一种长期风险。为了更好地描述这种风险，我们分析了 7079 名（年龄最大 21 岁）骨肉瘤或软组织肿瘤患者的数据。我们的研究结果为年轻肉瘤幸存者提供了关于其个体继发恶性肿瘤风险的依据。此外，这些数据有助于为年轻肉瘤幸存者制定后续护理建议。
