Göbel U, Bamberg M, Calaminus G, Gnekow A K, Herrmann H D, Lenard H G, Spaar H J, Niethammer D, Kühl J, Harms D
Universitäts-Kinderklinik Düsseldorf.
Klin Padiatr. 1993 Jul-Aug;205(4):217-24. doi: 10.1055/s-2007-1025230.
Germ cell tumors of the central nervous system are histological identical to the extracranial tumor sites. According to the localisation germ cell tumors of the CNS are different in symptoms, diagnostic approaches, kind and location of metastases and stratification of therapy. Since 1986 patients with intracranial germ cell tumors are registered in the ongoing study for non-testicular germ cell tumors (MAKEI) of the German Society of Pediatric Oncology and Hematology, and are treated in accordance to therapy guidelines for extracranial sites. In MAKEI 89 therapy strategy was revised with a reduction of radiotherapy and an increased cumulative cisplatinum dose from 200 mg/m2 to 400 mg/m2. Patients with germinoma receive after histologic diagnosis radiotherapy consisting of 30 Gy craniospinal irradiation and 15 Gy tumorboost. Malignant non-germinoma receive after diagnosis by tumor marker in CSF and/or serum 2 courses bleomycin 15 mg/m2 day 1-3, Etoposide 150 mg/m2 day 1 + 2 and cisplatinum 20 mg/m2 days 4-8 (BEP), continued by 2 courses Vinblastine 3 mg/m2 day 1 + 2, Ifosfamide 1500 mg/m2 days 1-5 and cisplatinum 20 mg/m2 days 1-5 (VIP), followed by 30 Gy craniospinal irradiation and 20 Gy tumor boost. In teratoma first line therapy is complete resection. In incomplete resected cases adjuvant chemotherapy according to histological grading is administered. Until 31st January, 1993 101 patients (pts) were registered, containing 69 protocol pts. Diagnosis in protocol pts was teratoma in 8 cases, 2 pts died postnatal because of extended disease, 2/8 pts relapsed, but were salvaged by chemotherapy. 40 pts offered germinomas.(ABSTRACT TRUNCATED AT 250 WORDS)
中枢神经系统生殖细胞肿瘤在组织学上与颅外肿瘤部位相同。根据定位不同,中枢神经系统生殖细胞肿瘤在症状、诊断方法、转移的类型和部位以及治疗分层方面存在差异。自1986年以来,颅内生殖细胞肿瘤患者被纳入德国儿科肿瘤学和血液学协会正在进行的非睾丸生殖细胞肿瘤研究(MAKEI),并按照颅外部位的治疗指南进行治疗。在MAKEI 89中,治疗策略进行了修订,减少了放疗剂量,并将顺铂累积剂量从200mg/m²增加到400mg/m²。生殖细胞瘤患者在组织学诊断后接受放疗,包括30Gy全脑全脊髓照射和15Gy肿瘤区加量照射。恶性非生殖细胞瘤患者在通过脑脊液和/或血清中的肿瘤标志物确诊后,接受2个疗程的博来霉素(15mg/m²,第1 - 3天)、依托泊苷(150mg/m²,第1 + 2天)和顺铂(20mg/m²,第4 - 8天)(BEP方案),接着是2个疗程的长春花碱(3mg/m²,第1 + 2天)、异环磷酰胺(1500mg/m²,第1 - 5天)和顺铂(20mg/m²,第1 - 5天)(VIP方案),随后是30Gy全脑全脊髓照射和20Gy肿瘤区加量照射。畸胎瘤的一线治疗是完整切除。在切除不完全的病例中,根据组织学分级给予辅助化疗。截至1993年1月31日,登记了101例患者,其中69例为符合方案患者。符合方案患者的诊断中,8例为畸胎瘤,2例患者出生后因疾病进展死亡,8例中有2例复发,但通过化疗挽救。40例为生殖细胞瘤。(摘要截选至250字)