原发中枢神经系统生殖细胞瘤诊治

原发中枢神经系统生殖细胞肿瘤(primary central nervous system germ cell tumors,CNS GCTs)好发于儿童青少年和年轻成人。主要位于松果体和鞍上区。病理上分为胚组织瘤(germinoma)和非胚组织瘤(Nongerminoma,NGGCT),后者包括畸胎瘤、胚胎癌、内胚窦瘤和绒毛膜上皮癌。治疗前明确病理诊断对治疗方案的选择非常重要。肿瘤标记物AFP和β-HCG的检测有助于鉴别诊断。胚组织瘤主要采用放射治疗,对儿童患者可通过联合化疗减少放疗的剂量和范围。非胚组织瘤对放疗敏感性较差,需要化疗、放疗或手术等综合治疗改善生存率。CNSGCTs具有侵袭性和转移性的行为,不合适单纯采用立体定向伽玛射线(伽玛刀)治疗。针对CNSGCTs的生物学特点,采用减少不良反应保证疗效的综合治疗已取得较好的结果,值得进一步研究。

Diagnosis and Treatment of Primary Central Nervous System Germ Cell Tumors

Primary central nervous system germ cell tumors(CNS GCTs)often occur in children, adolescents and young adults. It is mostly located in pineal region and suprasellar region. CNS-GCTS can be pathologically divided into germinomas and nongerminomatous GCTS, which includes teratoma, embryonal carcinoma, endodermal sinus tumor, and choriocarcinoma. Accurate pathological diagnosis is important in guidance of therapy. Detection of tumor markers is an useful and non-invasive test in the diagnostic exercise. The mainstay of treatment for germinoma is radiotherapy. Chemotherapy combined with reduced-dose and-volume radiotherapy should be used in children with germinoma. Nongerminomatous GCTS are less sensitive to radiotherapy. Chemotherapy combined radiotherapy or surgery would improve the survival rate for nongerminomatous GCT. CNS-GCTS are malignant tumors with invasive and metastatic behavior. Gamma knife radiosurgery alone is not suitable for these patients. According to biological characteristics of CNS-GCTS, comprehensive therapy with reduced side effects and satisfactory results warrants further study.