颅内非生殖细胞瘤性恶性生殖细胞肿瘤的分级诊治

Grading system for diagnosis and treatment of intracranial nongerminomatous malignant germ cell tumors

目的探讨颅内非生殖细胞瘤性恶性生殖细胞肿瘤（NGMGCT）的临床特点，治疗措施和预后。方法回顾性分析39例经病理学证实的颅内非生殖细胞瘤性恶性生殖细胞肿瘤。其中男33例，女6例，年龄2～40岁。根据Matsutani的分类法，将其分为预后中等组（35例）与预后不良组（4例），对各组的临床特点、诊断、治疗方法和预后进行分析。结果39例患者中混合性生殖细胞瘤15例，未成熟畸胎瘤15例，胚胎癌7例，卵黄囊瘤2例。肿瘤全切者29例，次全切除5例，部分切除3例，2例行穿刺活组织检查。共随访到34例（87．2％），总的5年生存率为36．8％。预后中等组与预后不良组的5年生存率分别为42．6％与0。预后中等组中术后化放疗结合治疗与术后生存率显著相关（P〈0．05）。其中未成熟畸胎瘤患者中术后接受伽玛刀治疗患者的生存率高于未接受伽玛刀治疗者（P〈0．01），前者5年生存率100％。结论NGMGCT可划分为预后中等组和预后不良组。两组预后明显不同（P〈0．01）。胚胎癌和未成熟畸胎瘤预后相似，均可归入预后中等组。术后化放疗结合伽玛刀治疗可以提高预后中等组生存率。

Objective To discuss the clinical feature, treatment and prognosis of intracranial nongerminomatous malignant germ cell tumors （NGMGCT）. Methods The records of 39 patients receiving treatment at our hospital between 1995 and 2007 were reviewed retrospectively. According to the classification of Matsutani, they were grouped into intermediate prognosis and poor prognosis groups based on tumor histology. Clinical manifestations, diagnosis, treatment and outcome were analyzed in each group. Results In these 39 cases, there were 15 mix germ cell tumors, 15 immature teratomas, 7 embryonal carcinomas and 2 yolk sac tumors. All patients were treated surgically. The tumor was totally removed in 29 cases, sub-totally in 5 and partially in 3. Biopsy was performed in the other 2 cases. Thirty-four patients （ 87.2% ） were followed up. The overall 5-year survival rate was 36.8%. The 5-year actuarial survival rate for patients in the intermediate prognosis and poor prognosis groups were 42. 6% and 0 respectively. Chemoradiotherapy had a significant correlation with the prognosis of intermediated prognosis group （P = 0. 039 ）. The 5-year survival rate of immature teratoma patients receiving post-operative gamma knife surgery was 100%. It had significant difference （ P = 0. 0049） as compared to the 5-year survival rate of patients receiving no gamma knife surgery. Conclusion NGMGCT can be divided into the intermediate and poor groups based on the prognosis （ P = 0. 0003 ）. Embryonal carcinoma can be classified to the intermediate prognosis group because of its similar prognosis with immature teratoma and mixed tumors composed mainly of germinoma or teratoma. Surgery remains the first choice for NGMGCT since treatment should be based on tumor histology. For patients in the intermediate prognosis group, a combined regimen of surgical resection, radiotherapy, chemotherapy and gamma knife surgery is mostly effective.