Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group
Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group
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DOI：
10.1023/a:1026579623136
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发表时间：
2000-11-01
影响因子：
50.5
通讯作者：
Carli, M
中科院分区：
文献类型：
--
作者：
Casanova, M;Ferrari, A;Carli, M
背景：腺泡状软组织肉瘤（ASPS）是一种罕见的恶性肿瘤，其临床特点和治疗方法尚不清楚。我们报告了一系列的19名儿科患者管理超过20 years.Patients和方法：原发性保守性手术在所有患者中进行，是根治性的9，非根治性的3，7例患者进行了单独活检（3不可切除的肿瘤，4转移性疾病）。在两例根治性手术后进行了初步化疗。结果：中位随访74个月，全组5年生存率为80%，其中局限性肿瘤为91%，≤ 5cm为100%，> 5cm为31%; 19例患者中有16例存活（12例首次持续缓解时肿瘤大体切除）。化疗取得了两个部分缓解7 evaluable patients.Conclusions：小儿ASPS有更有利的预后比成人同行。在这个系列中，肿瘤大小与发病时的转移性疾病相关，并且是影响生存的主要因素。手术是主要的治疗方法。辅助治疗的有效性仍有待确定，但在手术不充分的情况下，放射治疗可能是可取的。
Background: Alveolar soft part sarcoma (ASPS) is a rare malignant tumor and little is known about its clinical features and management. We report on a series of 19 pediatric patients managed over 20 years.Patients and methods: Primary conservative surgery was performed in all patients and was radical in nine, non-radical in three; seven patients underwent biopsy alone (3 unresectable tumors, 4 metastatic disease). In two cases radical surgery was performed after primary chemotherapy. Radiotherapy was delivered to 8 patients, chemotherapy to 15.Results: After a median follow-up of 74 months, the five-year survival was 80% for the whole series, 91% for patients with localized disease, 100% for patients with tumor less than or equal to5 cm, and 31% for those >5 cm; 16 of 19 patients were alive (12 of 12 with grossly-resected tumor in first continuous remission). Chemotherapy achieved two partial remission among seven evaluable patients.Conclusions: Pediatric ASPS has a more favorable prognosis than its adult counterpart. In this series, tumor size correlates with metastatic disease at onset and is the major factor influencing survival. Surgery is the mainstay of therapy. The effectiveness of adjuvant therapy remains to be established, though radiotherapy may be advisable in cases of inadequate surgery.