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儿童髓母细胞瘤放疗后迟发性闭塞性血管病变。两例报告。

Late postirradiation occlusive vasculopathy in childhood medulloblastoma. Report of two cases.

作者信息

Grenier Y, Tomita T, Marymont M H, Byrd S, Burrowes D M

机构信息

Division of Pediatric Neurosurgery, Children's Memorial Hospital, Northwestern University Medical School, Chicago, Illinois 60614, USA.

出版信息

J Neurosurg. 1998 Sep;89(3):460-4. doi: 10.3171/jns.1998.89.3.0460.

Abstract

The authors report two cases of ischemic stroke secondary to occlusive vasculopathy two decades after radiation therapy (RT) for medulloblastoma. Both patients underwent posterior fossa medulloblastoma partial resection, followed by craniospinal RT in which a cobalt 60 source was used; 40 Gy were given to the whole brain plus a 15-Gy boost to the posterior fossa. Both patients received multiagent chemotherapy, immediately following radiation therapy in the first case and after repeated craniotomy for recurrence 13 years after radiation in the second case. They experienced multiple sequelae from radiation and chemotherapy, including growth retardation and psychomotor delay. However, 20 years after treatment, they remained tumor free and able to work, until they presented with focal neurological deficits and seizures. Computerized tomography and magnetic resonance imaging of the brain in both cases showed no tumor recurrence, but did demonstrate ischemia in a posterior cerebral artery distribution. Cerebral angiography revealed multiple mid-sized arterial wall irregularities as well as focal stenoses consistent with a postirradiation vasculopathy. The pathophysiological mechanisms, radiological appearance, and incidence of this syndrome are reviewed from the literature.

摘要

作者报告了两例在髓母细胞瘤放射治疗(RT)二十年后继发于闭塞性血管病的缺血性中风病例。两名患者均接受了后颅窝髓母细胞瘤部分切除术,随后进行了颅脊髓放疗,其中使用了钴60源;全脑给予40 Gy,后颅窝额外给予15 Gy的增强剂量。两名患者均接受了多药化疗,第一例在放疗后立即进行,第二例在放疗13年后因复发进行反复开颅术后进行。他们经历了放疗和化疗的多种后遗症,包括生长发育迟缓以及精神运动发育迟缓。然而,治疗20年后,他们一直无肿瘤且能够工作,直到出现局灶性神经功能缺损和癫痫发作。两例患者的脑部计算机断层扫描和磁共振成像均未显示肿瘤复发,但确实显示了大脑后动脉分布区的缺血。脑血管造影显示多处中等大小动脉壁不规则以及与放疗后血管病相符的局灶性狭窄。本文从文献中回顾了该综合征的病理生理机制、影像学表现及发病率。

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