Contemporary Approach to Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumors.

Q1 Medicine

American Society of Clinical Oncology educational book / ASCO. American Society of Clinical Oncology. Meeting Pub Date : 2024-06-01 DOI:10.1200/EDBK_432242

Angela C Hirbe, Carina A Dehner, Eva Dombi, Vanessa Eulo, Andrea M Gross, Taylor Sundby, Alexander J Lazar, Brigitte C Widemann

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Abstract

Most malignant peripheral nerve sheath tumors (MPNSTs) are clinically aggressive high-grade sarcomas, arising in individuals with neurofibromatosis type 1 (NF1) at a significantly elevated estimated lifetime frequency of 8%-13%. In the setting of NF1, MPNSTs arise from malignant transformation of benign plexiform neurofibroma and borderline atypical neurofibromas. Composed of neoplastic cells from the Schwannian lineage, these cancers recur in approximately 50% of individuals, and most patients die within five years of diagnosis, despite surgical resection, radiation, and chemotherapy. Treatment for metastatic disease is limited to cytotoxic chemotherapy and investigational clinical trials. In this article, we review the pathophysiology of this aggressive cancer and current approaches to surveillance and treatment.

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神经纤维瘤病 1 型相关恶性周围神经鞘瘤的现代治疗方法。

大多数恶性周围神经鞘瘤（MPNSTs）是临床上侵袭性很强的高级别肉瘤，多发于 1 型神经纤维瘤病（NF1）患者，估计终生发病率高达 8%-13%。在 NF1 患者中，MPNST 是由良性丛状神经纤维瘤和边缘不典型神经纤维瘤恶性转化而来。这些癌症由 Schwannian 系的肿瘤细胞组成，约有 50% 的患者会复发，尽管进行了手术切除、放疗和化疗，但大多数患者仍会在确诊后五年内死亡。转移性疾病的治疗仅限于细胞毒性化疗和临床试验研究。在本文中，我们将回顾这种侵袭性癌症的病理生理学以及目前的监控和治疗方法。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

American Society of Clinical Oncology educational book / ASCO. American Society of Clinical Oncology. Meeting Medicine-Medicine (all)

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期刊介绍： The Ed Book is a National Library of Medicine–indexed collection of articles written by ASCO Annual Meeting faculty and invited leaders in oncology. Ed Book was launched in 1985 to highlight standards of care and inspire future therapeutic possibilities in oncology. Published annually, each volume highlights the most compelling research and developments across the multidisciplinary fields of oncology and serves as an enduring scholarly resource for all members of the cancer care team long after the Meeting concludes. These articles address issues in the following areas, among others: Immuno-oncology, Surgical, radiation, and medical oncology, Clinical informatics and quality of care, Global health, Survivorship.