儿童内脏非横纹肌肉瘤软组织肉瘤的治疗探讨

目的探讨儿童内脏非横纹肌肉瘤软组织肉瘤的诊治要点。方法2004年7月至2010年7月我们收治儿童内脏非横纹肌肉瘤软组织肉瘤10例，其中男性7例，女性3例，年龄最大12岁，最小2岁。结果10例患儿中，手术后病理类型包括滑膜肉瘤1例，婴儿型纤维肉瘤2例，促结缔组织增生性小圆细胞肿瘤（DSRCT）1例，淋巴管平滑肌肉瘤1例，腹膜后平滑肌肉瘤1例，大网膜脂肪肉瘤1例，原始神经外胚叶肿瘤3例。肿瘤直径均〉5cm，以手术、化疗或放疗综合治疗为原则，其中淋巴管平滑肌肉瘤1例，腹膜后平滑肌肉瘤1例，大网膜脂肪肉瘤1例手术完整切除，术后定期随访，未作放疗和化疗。其他7例手术后辅助化疗和放疗。所有病例最长随访时间7年，最短15个月，平均随访时间（41．3±23．19）个月。结论内脏非横纹肌肉瘤软组织肉瘤因部位及肿瘤大小而影响手术完整切除，提高手术切除的技能、寻找更加有效的化疗和放疗是内脏NRSTS今后治疗的方向。

The management of visceral non-rhabdomyosarcoma soft tissue sarcomas in children.

Objetive To explore the diagnosis and treatment of visceral non-rhabdomyosarcoma soft tis- sue sarcoma in children. Methods Between 2004.7 -2010.7 , 10 cases of non-rhabdomyosarcoma soft tissue sarcoma were treated in Shanghai Children Medical Center, 7 were males and 3 females, the maximum age was 12 years , the minimum age was 2 years. Results After surgery, pathological type including 1 case of synovial sarcoma , 2 cases of infantile fibrosarcoma, 1 case of small round cell tumor （DSRCT） , 1 case of lymphatic leiomyosarcoma, 1 case of retroperitoneai ieiomyosarcoma , 1 case of hposarcoma of the omentum, 3 cases of primitive neuroectodermal tumor. Tumor size all greater than 5cm. treatment principle is surgery com- bine with chemotherapy and radiotherapy. 3 cases （lymphatic leiomyosarcoma,retroperitoneal leiomyosarcoma and liposarcoma of the omentum） treatment emphasizes complete surgical and postoperative follow-up on a regu- lar , did not make any radiotherapy and chemotherapy. The others surgery after neoadjuvant chemotherapy and radiotherapy. Long-term follow-up of all cases, the maximum time was seven years, the minimum time was 15 months. Conclusion Patients with visceral NRSTS had worse outcome, improve the skills of surgical resec- tion, looking for more effective chemotherapy and radiotherapy is one of visceral NRSTS future treatment direc- tions.