椎管内、颅内多发性原始神经外胚叶肿瘤一例报告并文献复习

Multiple intraspinal and intracranial primitive neuroectodermal tumors （ PNETs ） case report and review of literature

目的报告椎管内、颅内多发性原始神经外胚叶肿瘤1例，进行文献复习，并对其治疗学及预后进行探讨。方法1例椎管内、颅内多发性原始神经外胚叶肿瘤，先后共进行5次椎管探查肿瘤切除术，1次脑积水脑室-腹腔分流术、2次全神经轴常规放疗及2次头颅X-刀治疗第四脑室、颞极占位。结果5次椎管探查均达到肿瘤镜下全切除，术后症状均有不同程度缓解，未出现新的神经功能缺失；分流术后脑积水消失；X-刀治疗后第四脑室占位消失；全神经轴放疗后40个月发现椎管肿瘤再次复发。随访78个月，死于泌尿系感染、肾功能衰竭。结论椎管内、颅内多发性原始神经外胚叶肿瘤极为罕见，预后差。应用显微外科技术尽量全切肿瘤结合术后正规放疗有利于延长患者生存期。

Objective To study the treatment of multiple intraspinal and intracranial primitive neuroectodermal tumors（PNETs） by reporting a case and reviewing the literatures. Method To report a case of multiple intraspinal and intracranial PNETs in which 5 intraspinal surgery of tumors resection ,2 times of routine radiotherapy of brain and spinal cord, ventricle - operitoneal shunt for hydrocephalus, and twice X -knife radiotherapy for tumor in 4th ventricle and temperal lobe were performed. Results The microscopic total removal of spinal tumors were achieved in all 5 intraspinal operations. The preoperative symptoms were relieved and there was no new postoperative neurological defect. The hydrocephalus disappeared after shunt operation. The 4th ventricle tumor disappeared after X - knife radiotherapy. The recurrence of spinal tumor was observed 40 months after radiotherapy of brain and spinal cord. The patient died of renal failure after 78 months follow- up. Conclusions Multiple intraspinal and intracranial PNETs is a really rare condition which may be with poor prognosis. Microscopic total resection of tumors combined with postoperative normalized radiotherapy is beneficial to prolong the living period of patient.