Radiation Therapy in Management of Sporadic and Neurofibromatosis Type 1-associated Malignant Peripheral Nerve Sheath Tumors

Overview

Journal Front Oncol

Specialty Oncology

Date 2014 Dec 3

PMID 25452937

Citations 49

Authors

Jenna Kahn

Andy Gillespie

Maria Tsokos

John Ondos

Eva Dombi

Kevin Camphausen

Brigitte C Widemann

Aradhana Kaushal

Affiliations

Soon will be listed here.

Abstract

Introduction: Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive soft tissue sarcomas in which complete surgical resection is the mainstay of therapy. However, the recurrence rate is high and few options remain for refractory or metastatic MPNST. This study examines the outcomes of adjuvant radiation therapy in MPNST in patients with and without neurofibromatosis type 1 (NF1) and reviews the literature on use of radiation for MPNST.

Methods: A retrospective review of 33 MPNST patients between 1990 and 2012 evaluated at the NIH. All diagnoses were pathologically confirmed at the NCI. Clinical presentation, treatment, and survival were analyzed.

Results: Thirty-three patients were included 18 NF1-associated, 15 sporadic tumors. Tumor location included extremity (58%), trunk (36%), and head/neck (6%). Histologic grade showed 25 high-grade tumors compared to 7 low-grade tumors. Twenty patients were treated with radiation therapy (median total dose of 58.5 Gy with 1.8 Gy/fraction). A median survival of all patients was 46.5 months and 43.7% overall 5-year survival. Prognostic factors include extent of resection, tumor location, and histology grade. Radiation was not found to be a prognostic factor for overall survival.

Conclusion: This study is consistent with previous studies regarding the role of radiation in the management of MPNST. Prospective evaluation of adjuvant radiation will allow to more fully define the role of radiation in MPNST.

Citing Articles

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Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review.

Skorka P, Kordykiewicz D, Ilkow A, Ptaszynski K, Wojcik J, Skorka W Life (Basel). 2025; 14(12.

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[Analysis of clinical features, treatment methods, and prognostic influence factors in patients with malignant peripheral nerve sheath tumor].

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Clinical Outcomes of Carbon Ion Radiation Therapy for Malignant Peripheral Nerve Sheath Tumors.

Fiore M, Chalaszczyk A, Barcellini A, Vitolo V, Fontana G, Russo S Adv Radiat Oncol. 2024; 9(11):101619.

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