Two-year Local and Distant Control of Malignant Peripheral Nerve Sheath Tumor (MPNST) in NF1 Patients After Multiple Recurrences: A Case Report

Mostafa  Farzin; atemeh  Jafari; Ava  Yousefi

doi:10.18502/crcp.v9i2.16925

Mostafa Farzin Department of Radiation Oncology, Cancer Institute, Imam Khomeini Hospital complex, Tehran University of Medical Sciences, Tehran, Iran.
atemeh Jafari Department of Radiation Oncology, Cancer Institute, Imam Khomeini Hospital complex, Tehran University of Medical Sciences, Tehran, Iran.
Ava Yousefi Department of Radiation Oncology, Cancer Institute, Imam Khomeini Hospital complex, Tehran University of Medical Sciences, Tehran, Iran.

DOI: https://doi.org/10.18502/crcp.v9i2.16925

Keywords: MPNST; NF1; RT; Chemotherapy; Local control; Distant contro

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, biologically high-grade sarcomas that tend to recur and metastasize. The known risk factors for MPNSTs include neurofibromatosis type 1, highly aggressive MPNSTs, and dismal survival outcomes. Multimodality therapies, including surgical resection, radiotherapy, chemotherapy, targeted therapy, and combination therapy, are available. This case report describes a young woman with NF1 who was referred to us with a history of three local recurrences of MPNSTs in the proximal part of her left thigh after several surgical attempts. Successful local and distant control was achieved via preoperative radiotherapy and chemotherapy prior to the latest surgery. With preoperative RT and chemotherapy, more long-term and successful local and distant control was achieved, particularly in high-risk NF1- associated MPNST patients with a history of recurrence. Trials with larger sample sizes may show improvements in local control (LC), disease-free survival (DFS), and overall survival (OS) with neoadjuvant interventions in such patients.