Neural Tumors Program, Center for Cancer and Blood Diseases, Childrens Hospital Los Angeles, Los Angeles, California, USA.
Pediatr Blood Cancer. 2010 Jul 15;55(1):42-6. doi: 10.1002/pbc.22468.
BACKGROUND: The purpose of this study was to evaluate a reduced irradiation dose strategy for newly diagnosed primary central nervous system (CNS) germinomas. METHODS: Twenty patients with histologically diagnosed localized pure germinoma (n = 19) or germinoma with a mature teratoma component (n = 1) received four cycles of carboplatin and etoposide at 3-week intervals. In 18 patients, chemotherapy was followed by whole ventricular irradiation to 21.6-25.5 Gy with a simultaneous integrated or sequential primary site boost to 30-30.6 Gy. Initial tumor markers for beta-human chorionic gonadotrophin (HCGbeta) and alpha-fetoprotein (AFP) were evaluated in serum and lumbar cerebrospinal fluid (CSF). Endoscopic biopsies were performed in 12 patients and partial resections in the remaining 8 patients at diagnosis. Neurocognitive function was evaluated periodically following treatment. RESULTS: Eighteen of 20 patients are without evidence of residual or recurrent tumor. Both relapsing patients were subsequently determined to harbor malignant non-germinomatous germ cell tumor (NGGCT). This retrospective study shows that the Kaplan-Meier estimates of event-free survival (EFS) and overall survival (OS) at 3 years for all 20 patients were 89.5 +/- 7.1% and 100%, respectively. Neurocognitive function was well preserved in all 19 evaluable patients. CONCLUSION: Chemotherapy followed by reduced dose whole ventricular and local boost irradiation appears to be effective in patients with localized pure CNS germinoma with evidence of preservation of neurocognitive function.
背景:本研究旨在评估新诊断原发性中枢神经系统(CNS)生殖细胞瘤的低剂量照射策略。
方法:20 例组织学诊断为局限性纯生殖细胞瘤(n=19)或生殖细胞瘤伴成熟畸胎瘤成分(n=1)的患者接受 4 个周期的卡铂和依托泊苷,每 3 周一次。在 18 例患者中,化疗后采用全脑室照射 21.6-25.5Gy,同时对原发部位进行同步综合或序贯局部加量至 30-30.6Gy。初始肿瘤标志物β-人绒毛膜促性腺激素(β-HCG)和甲胎蛋白(AFP)在血清和腰椎脑脊液(CSF)中进行评估。12 例患者在诊断时进行了内镜活检,其余 8 例患者进行了部分切除。治疗后定期评估神经认知功能。
结果:20 例患者中有 18 例无残留或复发肿瘤证据。2 例复发患者随后被确定为恶性非生殖细胞瘤生殖细胞瘤(NGGCT)。这项回顾性研究表明,20 例患者的无事件生存(EFS)和总生存(OS)的 Kaplan-Meier 估计值在 3 年时分别为 89.5 +/- 7.1%和 100%。19 例可评估的患者神经认知功能均良好保留。
结论:化疗后行低剂量全脑室和局部加量照射对局限性纯 CNS 生殖细胞瘤患者有效,且神经认知功能得以保留。
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