Bisogno Gianni, Sotti Guido, Nowicki Yohann, Ferrari Andrea, Garaventa Alberto, Zanetti Ilaria, Favre Claudio, Schiavetti Amalia, Tamaro Paolo, Carli Modesto
Division of Hematology/Oncology, Department of Pediatrics, University Hospital of Padua, Padua, Italy.
Cancer. 2004 Apr 15;100(8):1758-65. doi: 10.1002/cncr.20159.
Survivors of childhood malignancies have an increased risk of developing second malignant neoplasms (SMN) due to their prior treatment and/or genetic susceptibility. A small proportion of SMNs are soft tissue sarcomas (STS), whose prognosis is generally thought to be poor, though publications on such patients' treatment and outcome is limited.
The authors analyzed 25 patients who were registered for the Italian Cooperative Group protocols for pediatric STS from 1979 to 2000. The primary tumor was STS in five patients; Hodgkin disease in five patients; leukemia in four patients; retinoblastoma, neuroblastoma, and Wilms tumor in two patients each; and other tumor types in five patients. SMNs occurred after a median of 8 years (range, 1.9-15.0 years) and included rhabdomyosarcoma (RMS) in 4 patients, malignant peripheral nerve sheath tumor in 4 patients, extraosseous Ewing family tumor (EFT) in 4 patients, leiomyosarcoma in 3 patients, fibrosarcoma in 2 patients, synovial sarcoma in 2 patients, and other tumor types in 6 patients. Treatment generally was administered according to the guidelines for primary STS.
Seven non-RMS patients with STS underwent surgery alone, whereas 18 patients received chemotherapy and 8 patients received radiotherapy. Retreatment was feasible with acceptable toxicity. Fifteen patients were alive in complete remission of their SMN at the time of last follow-up. Responses to chemotherapy and survival were satisfactory for patients with tumors such as RMS and EFT. Complete tumor resection was correlated with a favorable prognosis in patients with other types of STS and in patients with postirradiation sarcoma. Two patients developed a third malignancy.
Although prior treatment may hinder the management of these patients, pediatric STS second malignancies can be cured using the same strategies used for de novo pediatric sarcomas. Long-term follow-up is mandatory given the risks of further malignancies and more severe, treatment-related side effects.
儿童恶性肿瘤幸存者因先前的治疗和/或遗传易感性，发生第二原发性恶性肿瘤（SMN）的风险增加。一小部分SMN是软组织肉瘤（STS），尽管关于此类患者治疗和结局的出版物有限，但一般认为其预后较差。
作者分析了1979年至2000年登记参加意大利合作组小儿STS方案的25例患者。5例患者的原发性肿瘤为STS；5例为霍奇金病；4例为白血病；2例为视网膜母细胞瘤、神经母细胞瘤和肾母细胞瘤；5例为其他肿瘤类型。SMN发生的中位时间为8年（范围1.9 - 15.0年），包括4例横纹肌肉瘤（RMS）、4例恶性外周神经鞘瘤、4例骨外尤因家族肿瘤（EFT）、3例平滑肌肉瘤、2例纤维肉瘤、2例滑膜肉瘤和6例其他肿瘤类型。治疗一般按照原发性STS的指南进行。
7例非RMS的STS患者仅接受了手术，而18例患者接受了化疗，8例患者接受了放疗。再次治疗可行，毒性可接受。在最后一次随访时，15例患者的SMN处于完全缓解状态存活。对于RMS和EFT等肿瘤患者，化疗反应和生存情况令人满意。完全肿瘤切除与其他类型STS患者和放疗后肉瘤患者的良好预后相关。2例患者发生了第三种恶性肿瘤。
尽管先前的治疗可能会阻碍这些患者的管理，但小儿STS第二原发性恶性肿瘤可采用与小儿原发性肉瘤相同的策略治愈。鉴于有进一步发生恶性肿瘤和更严重的与治疗相关副作用的风险，必须进行长期随访。
