成人小儿类脑肿瘤

Sandra Fernandes Dias, Oliver Richards, Martin Elliot, Paul Chumas
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摘要

小儿脑肿瘤在病理类型、解剖部位、分子特征和可能的肿瘤驱动因素方面都与成人不同。虽然这些肿瘤通常发生在儿童时期,但也很少出现在成年患者身上,要么是新诊断出的肿瘤,要么是儿童肿瘤在患者转入成人服务后延迟复发的肿瘤。由于儿童类肿瘤在成人中的罕见性,有关这些肿瘤类型在成人中的文献通常仅限于小规模的病例系列,治疗决策通常基于儿童研究中的管理方案。然而,这些肿瘤的生物学特性往往不同于在儿童中发现的相同肿瘤。同样,成人患者往往无法忍受儿童使用的积极治疗方法的副作用,而这些治疗方法在成人中的疗效证据很少或根本没有。在本章中,我们将回顾文献并总结以下儿科肿瘤类型的临床、病理、分子特征和治疗反应:成髓细胞瘤、上胚瘤、颅咽管瘤、朝粒细胞性星形细胞瘤、腮腺下巨细胞星形细胞瘤、生殖细胞瘤、脉络丛肿瘤、中线胶质瘤和多形性黄细胞瘤,并重点介绍其与成人的不同之处。
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Pediatric-Like Brain Tumors in Adults.

Pediatric brain tumors are different to those found in adults in pathological type, anatomical site, molecular signature, and probable tumor drivers. Although these tumors usually occur in childhood, they also rarely present in adult patients, either as a de novo diagnosis or as a delayed recurrence of a pediatric tumor in the setting of a patient that has transitioned into adult services.Due to the rarity of pediatric-like tumors in adults, the literature on these tumor types in adults is often limited to small case series, and treatment decisions are often based on the management plans taken from pediatric studies. However, the biology of these tumors is often different from the same tumors found in children. Likewise, adult patients are often unable to tolerate the side effects of the aggressive treatments used in children-for which there is little or no evidence of efficacy in adults. In this chapter, we review the literature and summarize the clinical, pathological, molecular profile, and response to treatment for the following pediatric tumor types-medulloblastoma, ependymoma, craniopharyngioma, pilocytic astrocytoma, subependymal giant cell astrocytoma, germ cell tumors, choroid plexus tumors, midline glioma, and pleomorphic xanthoastrocytoma-with emphasis on the differences to the adult population.

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