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小儿和成人髓母细胞瘤患者的管理。

Management of pediatric and adult patients with medulloblastoma.

作者信息

Martin Allison M, Raabe Eric, Eberhart Charles, Cohen Kenneth J

机构信息

The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Bloomberg 11379, 1800 Orleans Street, Baltimore, MD, 21287, USA.

出版信息

Curr Treat Options Oncol. 2014 Dec;15(4):581-94. doi: 10.1007/s11864-014-0306-4.

Abstract

Approximately 70 % of newly diagnosed children with medulloblastoma (MB) will be classified as "standard risk": their tumor is localized to the posterior fossa, they undergo a near or gross total resection, the tumor does not meet the criteria for large cell/anaplastic histology, and there is no evidence of neuroaxis dissemination by brain/spine MRI and lumbar puncture for cytopathology. Following surgical recovery, they are treated with craniospinal radiation therapy with a boost to the posterior fossa or tumor bed. Adjuvant therapy for approximately 1 year follows anchored by the use of alkylators, platinators, and microtubule inhibitors. This approach to standard risk MB works; greater than 80 % of patients will be cured, and such approaches are arguably the standard of care worldwide for such children. Despite this success, some children with standard risk features will relapse and die of recurrent disease despite aggressive salvage therapy. Moreover, current treatment, even when curative causes life-long morbidity in those who survive, and the consequences are age dependent. For the 20-year-old patient, damage to the cerebellum from surgery conveys greater risk than craniospinal radiation; however, for the 3-year-old patient, the opposite is true. The challenge for the neuro-oncologist today is how to identify accurately patients who need less therapy as well as those for whom current therapy is inadequate. As molecular diagnostics comes of age in brain tumors, the question becomes how to best implement novel methods of risk stratification. Are we able to obtain specific information about the tumor's biology in an increasingly rapid and reliable way, and utilize these findings in the upfront management of these tumors? Precision medicine should allow us to tailor therapy to the specific drivers of each patient's tumor. Regardless of how new approaches are implemented, it is likely that we will no longer be able to have a single standard approach to standard risk medulloblastoma in the near future.

摘要

大约70%新诊断的髓母细胞瘤(MB)患儿将被归类为“标准风险”:他们的肿瘤局限于后颅窝,接受了近全切除或全切除,肿瘤不符合大细胞/间变组织学标准,且脑/脊柱MRI和腰椎穿刺细胞病理学检查没有神经轴播散的证据。术后恢复后,他们接受颅脊髓放疗,并对后颅窝或肿瘤床进行强化放疗。随后进行约1年的辅助治疗,主要使用烷化剂、铂类药物和微管抑制剂。这种针对标准风险MB的治疗方法是有效的;超过80%的患者将被治愈,并且这种方法可以说是全球此类患儿的标准治疗方案。尽管取得了成功,但一些具有标准风险特征的患儿仍会复发,并死于复发性疾病,尽管接受了积极的挽救治疗。此外,目前的治疗即使能治愈,也会给幸存者带来终身的发病率,且后果与年龄有关。对于20岁的患者,手术对小脑的损伤比颅脊髓放疗带来的风险更大;然而,对于3岁的患者,情况则相反。当今神经肿瘤学家面临的挑战是如何准确识别那些需要较少治疗的患者以及那些目前治疗不足的患者。随着分子诊断在脑肿瘤领域的成熟,问题变成了如何最好地实施新的风险分层方法。我们能否以越来越快速和可靠的方式获得有关肿瘤生物学的具体信息,并将这些发现应用于这些肿瘤的初始治疗中?精准医学应该使我们能够根据每个患者肿瘤的特定驱动因素来定制治疗方案。无论新方法如何实施,在不久的将来,我们可能不再能够采用单一的标准方法来治疗标准风险的髓母细胞瘤。

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