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复发性李-弗劳梅尼综合征相关脉络丛癌的成功治疗

Successful Treatment of Recurrent Li-Fraumeni Syndrome-related Choroid Plexus Carcinoma.

作者信息

McEvoy Matthew, Robison Nathan, Manley Peter, Yock Torunn, Konopka Kristine, Brown Robert E, Wolff Johannes, Green Adam L

机构信息

*Department of Pediatrics, University of Colorado School of Medicine †Center for Cancer and Blood Disorders, Children's Hospital Colorado, Aurora, CO ‡Pediatric Neuro-Oncology, Children's Hospital Los Angeles, Los Angeles, CA §Department of Pediatric Oncology, Dana-Farber Cancer Institute ∥Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA ¶Department of Pathology, University of Michigan School of Medicine, Ann Arbor, MI #Department of Pathology, UTHealth McGovern Medical School, Houston, TX **AbbVie, Chicago, IL.

出版信息

J Pediatr Hematol Oncol. 2017 Nov;39(8):e473-e475. doi: 10.1097/MPH.0000000000000965.

Abstract

The management of choroid plexus carcinoma (CPC) is challenging and multifaceted. Here, we discuss a 3-year-old girl with CPC and Li-Fraumeni syndrome who achieved full remission after surgery and chemotherapy, with radiation therapy spared. At recurrence, we used a novel, standard-dose cytotoxic chemotherapy regimen, focal proton radiation therapy, and targeted agents based on morphoproteomic analysis to achieve long-term survival. We highlight the rationale for our therapy at recurrence, as well as the risk-benefit analyses necessary in decision making for these patients. Our strategy may be effective in managing other patients with recurrent CPC and Li-Fraumeni syndrome.

摘要

脉络丛癌(CPC)的管理具有挑战性且涉及多方面。在此,我们讨论一名患有CPC和李-弗劳梅尼综合征的3岁女孩,她在手术和化疗后实现了完全缓解,未接受放射治疗。复发时,我们采用了一种新型的标准剂量细胞毒性化疗方案、局部质子放射治疗以及基于形态蛋白质组分析的靶向药物,以实现长期生存。我们强调复发时治疗的基本原理,以及这些患者决策中必要的风险效益分析。我们的策略可能对管理其他复发性CPC和李-弗劳梅尼综合征患者有效。

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