Palatal Malignant Peripheral Nerve Sheath Tumor: A Case Report and Review of Literature Publisher

Summary: Ever heard of malignant peripheral nerve sheath tumors (MPNST)? A rare case in the palate shows a potential link with neurofibromatosis type 1. How can we improve detection? #MPNST #Neurofibromatosis

Karimi A ; Derakhshan S ; Moradzadeh Khiavi M ; Hosseini SM
Source: Rare Tumors Published:2026

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare, aggressive soft tissue malignancy associated with neurofibromatosis type 1. Although MPNST most commonly occurs in the extremities and trunk, it is rare in the head and neck region and extremely rare in the oral cavity, including the palate. MPNSTs arise from Schwann cells, de novo or from benign neural tumors. MPNSTs often occur between the ages of 30 and 50 years closely associated with neurofibromas. The median survival rate of the patients is 46-58% over ten years. Diagnosis is based on medical history and clinical examination, and treatment includes surgery, radiotherapy, and sometimes chemotherapy. Prognosis varies depending on location, size, and metastasis. We report an elderly female with a MPNST of the palate presenting with an exophytic lesion with details of clinicoradiographic and histopathologic features and long-term follow-up. This report describes a rare case of palatal MPNST and reviews the relevant literature. © The Author(s) 2026. This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).