成人幕上原始神经外胚层肿瘤的诊治分析(附10例报道)

Diagnosis and treatment of supratentorial primitive neuroectodermal tumor in adult patients(report of 10 cases)

目的探讨成人幕上原始神经外胚层肿瘤(PNET)的临床特点、诊断及治疗方法。方法回顾性分析2011年11月至2015年12月收治的10例成人幕上PNET的临床资料,均行手术治疗,术后均行辅助放疗,辅助化疗5例。结果肿瘤全切除1例,近全切除6例,大部分切除3例。术后随访3~48个月;5例死亡。5例术后辅助单纯放疗,随访期间死亡3例,死亡时间为术后2、6、8个月;术后辅助放、化疗5例,随访期间死亡2例,死亡时间为术后12、22个月。1例额叶PNET,手术全切肿瘤,术后辅助放、化疗,随访期间未见复发。结论成人幕上PNET为颅内罕见肿瘤,恶性度高,易沿脑脊液播散,预后差;确诊依赖病理检查;采用手术、放疗、化疗、免疫治疗等综合治疗有助于延长患者生存期。

Objective To investigate the clinical features, diagnosis and treatment of supratentorial primitive neuroectodermal tumor （PNET） in the adult patients. Methods The clinical data of 10 adult patients with supratentorial PNET treated from November, 2011 to December, 2015 were analyzed retrospectively. All the patients underwent surgery and received adjuvant radiotherapy, and 5 patients received adjuvant chemotherapy after the surgery. Results The total tumor resection was achieved in 1 patient, subtotal in 6, greatly partial in 3. The following-up period ranged from 3 to 48 months. Five patients died 2, 6, 8, 12 and 22 months after the operation respectively. Of 5 patients received adjuvant radiotherapy, 3 patients died 2, 6 and 8 months after the surgery postoperatively and 2 survived. Of 5 patients received adjuvant radiotherapy and chemotherapy, 2 died 12 and 22 months after the operation respectively and 3 survived. The tumor did not recur during the following-up in 1 patient with frontal lobe PNET, who received total resection of the tumor and postoperative adjuvant radiotherapy and chemotherapy. Conclusions PNET is a rare intracranial tumor in adults, highly malignant and disseminated easily along with the cerebrospinal fluid. The prognosis is poor in the adult patients with PNET. The diagnosis depends on pathological and immunohistochemical examinations. The resection of the PNET by surgery and postoperative adjuvant radiotherapy, chemotherapy and immunotherapy are helpful to prolonging the survival time of the patients.