Hibi S, Naya M, Takaya K, Morimoto M, Kataoka Y, Todo S, Imashuku S
Division of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine.
Gan To Kagaku Ryoho. 1993 Jul;20(9):1249-52.
Alveolar soft part sarcoma (ASPS) is one of the soft tissue sarcomas characterized by metastasis early in the course of the disease, regardless of slow tumor growth. The ultimate clinical outcome is, therefore, very poor. To date, the efficacy of combination chemotherapy for this tumor has not been confirmed. We present a case of ASPS in an 8-year-old boy with bilateral pulmonary metastasis who received systemic combination chemotherapy (CPM/IFO/ACD/ADR/CD DP/VP-16 as induction, and VCR/ACD as maintenance therapy) for eleven months. The result showed that despite the prevention of tumor progression, such intensive chemotherapy could not cure the disease. Besides refinement of chemotherapy regimens, immunotherapy might be considered for ASPS.
肺泡软组织肉瘤（ASPS）是软组织肉瘤之一，其特征是在疾病过程早期发生转移，尽管肿瘤生长缓慢。因此，最终的临床结局非常差。迄今为止，这种肿瘤的联合化疗疗效尚未得到证实。我们报告一例8岁患有双侧肺转移的ASPS男孩病例，该男孩接受了11个月的全身联合化疗（诱导化疗采用CPM/IFO/ACD/ADR/CD DP/VP - 16，维持治疗采用VCR/ACD）。结果显示，尽管预防了肿瘤进展，但如此强烈的化疗并不能治愈该疾病。除了优化化疗方案外，对于ASPS可能还应考虑免疫治疗。
