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多药化疗后减少放疗剂量治疗的中枢神经系统生殖细胞瘤患儿的预后

Outcomes of children with central nervous system germinoma treated with multi-agent chemotherapy followed by reduced radiation.

作者信息

Cheng Sylvia, Kilday John-Paul, Laperriere Normand, Janzen Laura, Drake James, Bouffet Eric, Bartels Ute

机构信息

Department of Paediatrics, University of British Columbia, Vancouver, BC, Canada.

Department of Pediatrics, Division of Haematology/Oncology/BMT, B.C. Children's Hospital, 4480 Oak Street, B315, Vancouver, BC, V6H 3V4, Canada.

出版信息

J Neurooncol. 2016 Mar;127(1):173-80. doi: 10.1007/s11060-015-2029-1. Epub 2016 Jan 7.

Abstract

CNS germinomas have an excellent prognosis with radiation therapy alone. However, in children, volume and dose of CNS radiation are associated with neurocognitive and neuroendocrine sequelae. Our objective was to determine long-term outcomes of our cohort who received chemotherapy and reduced radiation. This retrospective cohort study analyzed treatment and outcome of intracranial germinoma patients consecutively treated at Sick Kids, Toronto, Canada, from January 2000 to December 2013. 24 children (13 male, 11 female; median age 13.36 years) were identified. Median follow up was 61 months (range 1-144 months). Tumor location was suprasellar (n = 9), bifocal (8), pineal (6), and basal ganglia (1). Three children showed dissemination on imaging. 2/24 had only elevated serum human chorionic gonadotropin, 3/24 only elevated lumbar cerebrospinal fluid (CSF) hCG, and 2/24 had both elevated serum and lumbar CSF hCG. 23/24 children completed treatment and received multi-agent chemotherapy followed by either ventricular radiation (2340-2400 cGy) (n = 9), ventricular radiation + boost (1600 cGy) (n = 8), whole brain (2340 cGy) (n = 3), focal (4000 cGy) (n = 2) or craniospinal radiation (2340 cGy) (n = 1). Five-year progression free and overall survival was 96 and 100 % respectively. 8/24 patients with ventricular radiation ± boost (2340/4000 cGy) displayed stable full scale intelligence quotient over a mean interval of 3 years following radiation, but showed declined processing speed. In this limited experience, excellent 5-year overall survival rates were achieved with chemotherapy followed by reduced whole ventricular radiation even if ventricular radiation was delivered without boost.

摘要

中枢神经系统生殖细胞瘤仅通过放射治疗就有良好的预后。然而,在儿童中,中枢神经系统放疗的体积和剂量与神经认知和神经内分泌后遗症有关。我们的目的是确定接受化疗和减少放疗的队列的长期结果。这项回顾性队列研究分析了2000年1月至2013年12月在加拿大多伦多病童医院连续治疗的颅内生殖细胞瘤患者的治疗情况和结果。确定了24名儿童(13名男性,11名女性;中位年龄13.36岁)。中位随访时间为61个月(范围1 - 144个月)。肿瘤位置为鞍上(n = 9)、双灶性(8)、松果体(6)和基底神经节(1)。3名儿童在影像学上显示有播散。24例中有2例仅血清人绒毛膜促性腺激素升高,24例中有3例仅腰椎脑脊液(CSF)hCG升高,24例中有2例血清和腰椎CSF hCG均升高。24例中有23例完成治疗,接受了多药化疗,随后进行脑室放疗(2340 - 2400 cGy)(n = 9)、脑室放疗+推量(1600 cGy)(n = 8)、全脑放疗(2340 cGy)(n = 3)、局部放疗(4000 cGy)(n = 2)或全脊髓放疗(2340 cGy)(n = 1)。5年无进展生存率和总生存率分别为96%和100%。24例接受脑室放疗±推量(2340/4000 cGy)的患者中有8例在放疗后的平均3年间隔期内全量表智商保持稳定,但处理速度有所下降。在这一有限的经验中,即使脑室放疗未进行推量,化疗后减少全脑室放疗也能实现出色的5年总生存率。

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