小儿原始神经外胚叶肿瘤6例临床分析

Clinical Analysis of Six Children With Primitive Neuroectodermal Tumors

目的提高对小儿原始神经外胚叶肿瘤的认识。方法回顾性分析我院自2000年1月至2004年12月间收治的6例原始神经外胚叶肿瘤患儿的年龄、性别、发病部位、临床症状、转移部位、病理特征和治疗方案及预后。结果6例患儿中除左上臂肿块患儿接受了扩大根治术目前尚存活外,其余5例患儿皆因肿块巨大,无法切除或切除困难(眼球后肿瘤患儿)而行肿块活检术,且均死亡,平均存活时间为10.3月。6例患儿均接受了不同方案的化疗,平均化疗时间为7.5月,均未接受放疗。结论原始神经外胚叶肿瘤是一种来源于神经外胚叶组织的小细胞恶性肿瘤,具有早期诊断困难,治疗棘手,死亡率高的特点。

Objective To heighten the realization of primitive neuroectodermal tumors in children. Methods Six cases of primitive neuroectodermal tumors in our hospital trom Jan.2000 to Dec.2004, the age, gender, the location of pathologic change, clinical symptoms, metastastic loca- tion,pathological characteristicsn, therapeutic regimen and prognosis were analyzed retrospectively. Results of six cases,except that one case whose tumor came from left upper arm underwent ex- tensuve radical resection, and is alive, other 5 cases only received biopsy due the tumors too huge volume to operation, and all of them died, the mean survival time was 10.3 months. All the 6 cases accepted different regimens of chemotherapy, mean chemotherapy was 7.5 monthes, but no radiotherapy. Conclusion Primitive neuroectoderrnal tumor is a malignant small cell tumor origin from neuroectoderm, it is characterized as difficulty to diagnose early as well as in treatment also is a knotty problem with high rate of mortality,