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成人中出现的典型儿科脑肿瘤——治疗与预后的差异

Typical Pediatric Brain Tumors Occurring in Adults-Differences in Management and Outcome.

作者信息

Greuter Ladina, Guzman Raphael, Soleman Jehuda

机构信息

Department of Neurosurgery, University Hospital of Basel, 4031 Basel, Switzerland.

Division of Pediatric Neurosurgery, University Children's Hospital of Basel, 4031 Basel, Switzerland.

出版信息

Biomedicines. 2021 Mar 30;9(4):356. doi: 10.3390/biomedicines9040356.

Abstract

Adult brain tumors mostly distinguish themselves from their pediatric counterparts. However, some typical pediatric brain tumors also occur in adults. The aim of this review is to describe the differences between classification, treatment, and outcome of medulloblastoma, pilocytic astrocytoma, and craniopharyngioma in adults and children. Medulloblastoma is a WHO IV posterior fossa tumor, divided into four different molecular subgroups, namely sonic hedgehog (SHH), wingless (WNT), Group 3, and Group 4. They show a different age-specific distribution, creating specific outcome patterns, with a 5-year overall survival of 25-83% in adults and 50-90% in children. Pilocytic astrocytoma, a WHO I tumor, mostly found in the supratentorial brain in adults, occurs in the cerebellum in children. Complete resection improves prognosis, and 5-year overall survival is around 85% in adults and >90% in children. Craniopharyngioma typically occurs in the sellar compartment leading to endocrine or visual field deficits by invasion of the surrounding structures. Treatment aims for a gross total resection in adults, while in children, preservation of the hypothalamus is of paramount importance to ensure endocrine development during puberty. Five-year overall survival is approximately 90%. Most treatment regimens for these tumors stem from pediatric trials and are translated to adults. Treatment is warranted in an interdisciplinary setting specialized in pediatric and adult brain tumors.

摘要

成人脑肿瘤大多与儿童脑肿瘤有所不同。然而,一些典型的儿童脑肿瘤在成人中也会出现。本综述的目的是描述成髓细胞瘤、毛细胞型星形细胞瘤和颅咽管瘤在成人和儿童中的分类、治疗及预后差异。成髓细胞瘤是一种世界卫生组织(WHO)IV级后颅窝肿瘤,分为四个不同的分子亚组,即音猬因子(SHH)、无翅型(WNT)、3组和4组。它们呈现出不同的年龄特异性分布,形成特定的预后模式,成髓细胞瘤在成人中的5年总生存率为25% - 83%,在儿童中为50% - 90%。毛细胞型星形细胞瘤是一种WHO I级肿瘤,在成人中大多位于幕上脑区,在儿童中则发生于小脑。完整切除可改善预后,其在成人中的5年总生存率约为85%,在儿童中>90%。颅咽管瘤通常发生在鞍区,通过侵犯周围结构导致内分泌或视野缺损。成人的治疗目标是大体全切,而在儿童中,保留下丘脑对于确保青春期内分泌发育至关重要。其5年总生存率约为90%。这些肿瘤的大多数治疗方案源于儿科试验并应用于成人。治疗应在专门针对儿童和成人脑肿瘤的多学科环境中进行。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/464a/8066180/922d21f417a5/biomedicines-09-00356-g001.jpg

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