颅内高度恶性非生殖细胞瘤性生殖细胞肿瘤

Intracranial nongerminomatous malignant germ cell tumors

目的探讨颅内高度恶性非生殖细胞瘤性生殖细胞肿瘤(non-germinomatous malignant germ cell tumors,NGMGCTs)的临床特点、治疗和预后。方法回顾性分析了19例高度恶性 NGMGCTs患者的临床特点、血清肿瘤标记物监测、治疗方法以及预后。其中胚胎癌2例,卵黄囊瘤4 例,绒毛膜上皮癌7例,混合性生殖细胞肿瘤6例。有7例术前肿瘤标记物监测均异常,所有患者均行手术切除肿瘤,全切除7例,近全切除10例,大部分切除2例。所有患者均行放疗,有11例行术后化疗。结果共随访到17例患者,平均随访3．5年,1年生存率为29．4％,3年生存率为5．9％。其中卵黄囊瘤1年生存率为25．0％;混合性生殖细胞肿瘤3年生存率为20．0％,胚胎癌和绒毛膜上皮癌患者皆于1年内死亡。结论高度恶性NGMGCTs预后很差。根据临床表现、影像学资料和肿瘤标记物甲胎蛋白(AFP)和(或)β-绒毛膜促性腺激素(β-HCG)增高,多数患者在术前可确诊为高度恶性NGMGCTs。治疗应先采用化疗后放疗,对治疗后肿瘤标记物水平降至正常而影像学上肿瘤仍未完全消失者,可考虑手术切除,术后再行化疗和(或)放疗。治疗结束后注意随诊,如肿瘤标记物再次升高,经影像学证实肿瘤复发,可再行化疗和手术治疗。这样会延长高度恶性NGMGCTs患者的生存期。

Objective To discuss the clinical features, treatment, and prognosis of intracranial nongerminomatous malignant germ cell tumors（ NGMGCTs ）. Methods The clinical data, serum levels of tumor markers,treatment regimens and prognosis of 19 patients with intracranial NGMGCTs were reviewed retrospectively. The pathological diagnosis was embryonal carcinoma in 2 patients, yolk sac tumor in 4, choriocarcinoma in 7, and mixed germ cell tumor containing these malignant components in 6 patients. There were 7 patients with elevated concentrations of serum. Among the 19 patients, the tumor was totally removed in7 cases, subtotally in10, and partially in 2. All patients received radiation therapy, and 11 patients received chemotherapy after surgery. Results Seventeen patients were followed-up with average 3.5 years, the 1- and 3- year survival rates were 29.4% and 5.9% respectively. The 1- year survival rates of patients with embryonal carcinoma, choriocarcinoma and yolk sac tumor were 0% , 0% and 25.0%. The 3- year survival rates of patients with yolk sac tumor and mixed tumors were 0% and 20. 0% respectively. Conclusions The prognosis of the 17 patients with intracranial NGMGCTs is very poor. Most NGMGCTs can be diagnosed based on clinical and imaging features, especially on elevated levels of serum tumor markers such as alpha- fetoprotein （AFP） and beta human chorionic gonadotrophin （β- HCG） before treatment. Chemotherapy followed by radiotherapy should be the first choice for these patients, Delayed surgical resection followed by chemotherapy and /or radiotherapy should be considered in patients with residual radiographic abnormalities and normalized tumor markers. Regular tests for tumor markers are strongly recommended after treatments, chemotherapy and surgery should be performed again for the patients with elevated levels of tumor markers and radiographic abnormalities. These treatment regimens may prolong survival duration for these patients with intracranial NGMGCTs.