Sawamura Y, Kato T, Ikeda J, Murata J, Tada M, Shirato H
Department of Neurosurgery, Hokkaido University School of Medicine, Sapporo, Japan.
J Neurosurg. 1998 Nov;89(5):728-37. doi: 10.3171/jns.1998.89.5.0728.
The optimum clinical management of central nervous system (CNS) teratomas, particularly postsurgical adjuvant therapy, is still unclear, partly as a result of the tumors' low incidence. In this study the authors analyze 34 cases of CNS teratomas so that they may adequately indicate management of these lesions.
The median age of the 34 patients was 13 years. Twenty-seven patients treated between 1970 and 1991 were retrospectively reviewed. Four of these 27 patients died as a result of radical surgery; each of them had a teratoma involving the hypothalamus. After initial treatment, which included radiation therapy, 20 patients (48%) had died. In all seven cases of mature teratomas there was no recurrence. In two cases of immature teratomas in which there was complete surgical resection there was recurrence; however, salvage therapies were effective. Seven of eight patients with highly malignant teratomas died; for these patients salvage therapies, including repeated radiation and chemotherapy, failed. Seven patients who presented with CNS teratomas between 1992 and 1996 received adjuvant chemotherapy and radiation therapy according to a prospective study protocol. All seven patients were free from recurrence with a 70 to 100% Karnofsky Performance Scale score at a median follow-up period of 41 months. Patients with CNS teratomas rarely responded completely to chemotherapy or radiation therapy; an effective adjuvant therapy produced a partial response at best.
Because teratomas show various responses to adjuvant therapy, a misdiagnosis of their histological subtype will lead to inadequate therapy. A diverse therapeutic protocol based on histological diagnosis is necessary to plan appropriate management. Treatment recommendations are discussed in detail in the article.
中枢神经系统（CNS）畸胎瘤的最佳临床管理，尤其是术后辅助治疗，仍不明确，部分原因是该肿瘤发病率较低。在本研究中，作者分析了34例CNS畸胎瘤病例，以便充分说明这些病变的管理方法。
34例患者的中位年龄为13岁。对1970年至1991年间接受治疗的27例患者进行了回顾性分析。这27例患者中有4例因根治性手术死亡；他们均患有累及下丘脑的畸胎瘤。在包括放射治疗在内的初始治疗后，20例患者（48%）死亡。7例成熟畸胎瘤均无复发。2例未成熟畸胎瘤行手术全切后复发；然而，挽救性治疗有效。8例高度恶性畸胎瘤患者中有7例死亡；对这些患者，包括重复放疗和化疗在内的挽救性治疗均失败。1992年至1996年间出现CNS畸胎瘤的7例患者根据前瞻性研究方案接受了辅助化疗和放疗。所有7例患者均无复发，在中位随访期41个月时卡氏评分70%至100%。CNS畸胎瘤患者很少对化疗或放疗完全反应；有效的辅助治疗最多只能产生部分反应。
由于畸胎瘤对辅助治疗表现出不同反应，对其组织学亚型的误诊将导致治疗不足。基于组织学诊断的多样化治疗方案对于规划适当的管理是必要的。本文详细讨论了治疗建议。
