椎管内髓外原始神经外胚层肿瘤临床病理特点分析

Clinical manifestation and pathological characteristics of intraspinal extramedullary peripheral primitive neuroectodermal tumour

目的分析椎管内髓外原始神经外胚层肿瘤（PNET）临床病理特点。方法11年余共收治5例椎管内髓外PNET患者，男3例，女2例，年龄11～48岁。全部原发于椎管内硬膜外，其中颈椎水平2例（C5～C7、C5～T1），胸椎4例（T4～T6、T6～T8、T4～T5、C5～T1）。主要临床症状表现为5例肢体麻木、活动障碍，4例大小便障碍。5例均有病理证实。结果3例患者病理行CD99检测，均为CD99（＋）。5例均先行手术切除后行术后放疗，其中4例并用术后化疗。5例治疗失败模式最先均为局部失败。5例患者生存时间（从治疗开始至死亡日期）分别为4．8、2．5、2．1、1．6、0．8年。结论椎管内髓外PNET是高度恶性肿瘤，生存期短，首次失败多出现在局部，治疗中应寻求提高肿瘤局部控制以获得更长缓解时间方案。pPNET病情进展到远处转移后生存期短，提示需全身化疗，但缺乏有效化疗方案。正确区分中枢性或外周性PNET对临床治疗方案选择及预后具有指导意义。

Objective To observe the clinical manifestation and pathological immunohistochemical features of intraspinal extramedullary peripheral primitive neuroectodermal tumour （PNET）. Methods From Apr. 1996 to Aug. 2007 ,totally 5 patients with intraspinal extramedullary peripheral PNET were treated,including 3 male and 2 female, aging from 11 to 48 years. The primary site was cervical vertebral in 2 （C5-C7 and CS-T1 ） and thoracic vertebral in 4 （T4-T6, T6-T8 ,T4-T5 and C5-T1 ）. The clinical manifestation was paresis of limbs in 5 and incontinence in 4. All the patients were pathologically diagnosed Results All of the three patients who had CD99 staining expressed CD99 （ ＋ ）. All the 5 patients received surgery followed by radiotherapy and 4 received postoperative chemotherapy. The most common treatment failure was local recurrence. The overall survival time of the 5 patients was 4.8year,2.5year,2. lyear,1.6year and 0.8 year respectively. Conclusions Intraspinal extramedullary peripheral PNET is highly malignant tumor. Patients have very poor survival and local control. New treatment protocol should be studied to improve the local disease control and increase remission rate. To distinguish central PNET from peripheral PNET has crucial significance since different entities have different treatment protocol and prognosis.