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(目的)探讨小儿恶性雪旺氏细胞瘤的发病特点及诊治方法。(方法)分析1988年6月至1997年10月收治的11例小儿恶性雪旺氏细胞瘤病例的临床病理特征、诊治方法及预后。(结果)颈部3例,腹部6例,纵隔及肩胛后各1例。其中3例为神经纤维瘤病变。均手术治疗,部分病例会并化、放疗。仅1例术后无瘤生存14个月,其余均在术后1-15个月死于肿瘤转移、局部浸润及相关并发症。(结论)小儿恶性雪旺氏细胞瘤发病不同于成人。辅助化、放疗有一定疗效,但预后不佳,迫切需要寻找新的综合治疗方法。
Objective To investigate the incidence and diagnosis and treatment of malignant Schwann cell tumor in children. (Method) The clinicopathological features, diagnosis and treatment methods and prognosis of 11 cases of malignant Schwannoblastoma in children admitted from June 1988 to October 1997 were analyzed. Results There were 3 cases of neck, 6 cases of abdomen and 1 case of mediastinal and posterior scapular. Three of them were neurofibromatosis lesions. All surgical treatment, some cases will be merged, radiotherapy. Only 1 patient survived 14 months after operation, and the rest died of tumor metastasis, local infiltration and related complications 1-15 months after operation. (Conclusion) The incidence of malignant Schwannoma in children is different from that in adults. Assisted, radiotherapy have a certain effect, but the prognosis is poor, there is an urgent need to find a new comprehensive treatment.