Adult-type soft tissue sarcomas in pediatric-age patients: Experience at the Istituto Nazionale Tumori in Milan
Adult-type soft tissue sarcomas in pediatric-age patients: Experience at the Istituto Nazionale Tumori in Milan
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DOI：
10.1200/jco.2005.02.053
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发表时间：
2005-06-20
影响因子：
45.3
通讯作者：
Fossati-Bellani, F
中科院分区：
文献类型：
--
作者：
Ferrari, A;Casanova, M;Fossati-Bellani, F
目的 非横纹肌肉瘤软组织肉瘤是一组异质性肿瘤，最佳治疗方法仍存在争议。我们报告了 182 名 18 岁以下的患者在 25 年的时间里在单一机构接受治疗的大组病例。患者和方法 在这个相对同质的成人型组织型亚组中，手术是主要的治疗方法。 73 例患者接受放疗，114 例患者接受化疗（其中 70 例接受化疗作为辅助治疗）。 结果 诊断时接受完全切除的患者的 5 年总生存率为 89%，边缘切除的患者为 79%，最初未切除的患者为 52%，发病时发生转移的患者为 17%。对于大型和高级别肿瘤的患者，即使进行了大体切除，结果也并不令人满意；辅助化疗似乎改善了该组的结果。最初未切除的患者对化疗反应良好，随后接受完全切除，无事件生存率约为 70%。对化疗的反应率为 58 %。结论 预后变量的识别应该能够规划风险适应治疗。最初患有无法切除的疾病的患者以及切除了大的和高级别肿瘤的患者发生转移和治疗失败的风险很高。尽管这种回顾性分析的局限性是不言而喻的，但我们的数据表明，强化化疗（使用异环磷酰胺-阿霉素方案）可能对这些患者产生比一般假设更重要的作用。
Purpose Nonrhabdomyosarcoma soft tissue sarcomas are a heterogeneous group of tumors for which optimal treatment remains controversial. We report on a large group of 182 patients younger than 18 years old treated at a single institution over a 25-year period.Patients and Methods In this relatively homogeneous subgroup of adult-type histotypes, surgery was the mainstay of treatment; radiotherapy was administered to 73 patients, and chemotherapy was administered to 114 patients (70 received chemotherapy as adjuvant therapy).Results Overall survival at 5 years was 89 % in patients who underwent complete resection at diagnosis, 79 % in patients who had marginal resection, 52 % in initially unresected patients, and 17 % in patients with metastases at onset. Outcome was unsatisfactory in patients with large and high-grade tumors, even after gross resection; adjuvant chemotherapy seemed to improve the results in this group. Initially unresected patients who responded well to chemotherapy and subsequently underwent complete resection had an event-free survival rate of approximately 70 %. The rate of response to chemotherapy was 58 %.Conclusion The identification of prognostic variables should enable risk-adapted therapies to be planned. Patients with initially unresectable disease and patients with resected large and high-grade tumors are at high risk of metastases and treatment failure. Although the limits of this retrospective analysis are self-evident, our data would suggest that intensive chemotherapy (with an ifosfamide-doxorubicin regimen) might have a more significant role in these patients than what is generally assumed.