Giant Epithelioid Malignant Perineural Nerve Sheath Tumor of the Head and Neck: A Case Report

Niloufar  Shabani; Maryam  Akbari; Seyede Misha  Seyednia; Ebrahim  Karimi; Farrokh  Heidari

doi:10.18502/acta.v64i1.21039

Niloufar Shabani Otorhinolaryngology Research Center, Tehran University of Medical Sciences, Tehran, Iran
Maryam Akbari Department of Otolaryngology and Head and Neck Surgery, Otorhinolaryngology Research Center, School of Medicine, Amiralmomenin Hospital, Guilan University of Medical Sciences, Rasht, Iran
Seyede Misha Seyednia Anesthesiology and Intensive Care, Amir A'lam Hospital, Tehran University of Medical Sciences, Tehran, Iran
Ebrahim Karimi Otorhinolaryngology Research Center, Tehran University of Medical Sciences, Tehran, Iran
Farrokh Heidari Otorhinolaryngology Research Center, Tehran University of Medical Sciences, Tehran, Iran

DOI: https://doi.org/10.18502/acta.v64i1.21039

Keywords: Ki-67 antigen; NF1

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive soft tissue sarcoma, with the epithelioid subtype accounting for less than 5% of cases. Most MPNSTs are associated with neurofibromatosis type 1 (NF1), prior radiation exposure, or occur sporadically. Head and neck involvement is uncommon, especially in large tumors. We report a case of giant sporadic epithelioid MPNST in a 21-year-old male without NF1. The patient presented with a progressively enlarging left head and neck mass over two years. Imaging revealed a heterogeneous lesion with extensive soft tissue, bony, and neural involvement. Histopathology confirmed epithelioid MPNST with high-grade spindle cell sarcoma features, positive for S-100 and p53, and a Ki-67 index of 20%. The patient underwent wide local excision with neurosurgical assistance, preserving major cranial nerves but requiring sacrifice of the facial nerve and jugular vein. Postoperative recovery was uneventful, and no recurrence was observed at six months. This case highlights the diagnostic complexity, aggressive nature, and surgical challenges of giant epithelioid MPNST in the head and neck, underscoring the importance of complete excision with negative margins for improved outcomes.