青少年下肢高分级软组织肉瘤的介入与手术治疗

Experience and discussion on treatment of high-grade soft tissue sarcomas of crura in yout

目的 探讨青少年下肢高分级软组织肉瘤介入化疗后行手术治疗的临床效果。方法 本组 6例患者年龄 12～ 2 3岁 ,男女各 3例 ,肿瘤位于小腿 4例、窝 1例、大腿 1例 ,最大径 4～ 15cm ,中位数 6cm。 6例患者均于术前行介入化疗 2次 ,化疗药物为长春地辛 4mg或长春新碱 2mg +顺氯氨铂 4 0～ 6 0mg +表阿霉素 5 0～ 80mg。 1例右侧小腿小圆细胞肉瘤患者在介入化疗间行静脉化疗 1次 ,1例右侧小腿原始神经外胚层肿瘤患者采用介入化疗、Co60 局部放疗、静脉化疗 1次及肿块广泛切除术 ,1例左侧大腿高分级软组织肉瘤患者介入化疗后行左股后肌间室切除术 ,余 3例行介入化疗及肿块广泛切除术 ,病理诊断分别为小腿腺泡状横纹肌肉瘤、窝滑膜肉瘤、小腿腺泡状软组织肉瘤。结果 介入化疗后肿块最大径缩小 1～ 8cm ,其中缩小 8cm者系肿块完全消失。 6例患者均成功保全肢体 ,下肢行走及运动功能无障碍。 6例患者均于介入后出现轻度患肢水肿及局部胀痛 ,1例并发下肢毛细淋巴管炎 ,远期患肢局部皮肤色素沉着。随访 6～ 2 0个月 ,患者均存活 ,1例于术后 2个月发现肺部阴影 ,局部无复发。结论 青少年下肢高分级软组织肉瘤行介入化疗可使肿块缩小 。

Objective To summarize the experience and investigate better method on treatment of high-grade soft tissue sarcomas of crura in youth. Methods Six young cases suffered from high-grade soft tissue sarcomas of crura were treated by interpolatory chemotherapy using vindesine 4mg or vincristine 2 mg combined with cisplatin 40-60 mg and 4′-Epidoxorubicin (4′-epi-ADM) 50-80 mg. There were 3 males and 3 females, and their ages rang from 12 to 23. The primary tumor locations were: calf in 4, thigh in 1, popliteal fossa in 1. The pathological type of these 6 cases were : 1 small round cell tumor from soft tissue, one rabdomyosarcoma, one alveoliar soft part sarcoma, one synovial sarcoma, one primitive neuroectodermal tumor and one without classification . All cases received interpolatory chemotherapy twice. Before their receiving interpolatory chemotherapy they had no tumor metastases. The maximum diameters of tumors rang from 4-15 cm. One patient′s tumor disappered after interpolatory chemotherapy and she received no further therapy. The other 5 cases received operations and one of them received additional pre-operational radiotherapy using Co^(60) and pre-operational systemic chemotherapy using ifosfamide and dacarbazine. Results The tumors dwindled in size of 1-8 cm after interpolatory chemotherapy. All cases saved their crura and had normal functions. After interpolatory chemotherapy, all patients suffered from edema and ache of crura. One patient suffered from lymphatitis. All these patients were followed up for 6-20 months . All of them were alive and had pigmentations on their crura. A lung metastasis was found in 1 patient by CT 2 months after surgery, then he was in hospital and treated by chemotherapy. Conclusions For treatment of high-grade soft tissue sarcomas of crura which are difficult to resect in youth , interpolatory chemotherapy is a good choice .