Radiation therapy in management of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors

Jenna Kahn, Andy Gillespie, Maria Tsokos, John Ondos, Eva Dombi, Kevin Camphausen, Brigitte C. Widemann, Aradhana Kaushal

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Article number324
JournalFrontiers in Oncology
Volume4
Issue numberNOV
DOIs
StatePublished - 2014

Keywords

  • Malignant peripheral nerve sheath tumor
  • Malignant schwannoma
  • Neurofibrosarcoma
  • Neurogenic sarcoma
  • Radiation therapy

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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    Kahn, J., Gillespie, A., Tsokos, M., Ondos, J., Dombi, E., Camphausen, K., Widemann, B. C., & Kaushal, A. (2014). Radiation therapy in management of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Frontiers in Oncology, 4(NOV), [324]. https://doi.org/10.3389/fonc.2014.00324