Clinicopathological and Therapeutic Challenge: A Case Report of a Malignant Peripheral Nerve Sheath Tumor

Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas associated with Schwann cells and neurofibromatosis type 1. The cutaneous subtype of MPNST is diagnosed primarily through histological findings, but immunohistochemistry is limited because of the overlap of markers with other soft tissue tumors. Treatment involves surgical excision followed by radiotherapy. Herein, we report a 37-year-old female patient who presented with a progressive, painless, cutaneous lesion in the left frontal region. Histopathological and immunohistochemical analyses revealed spindle cell neoplastic proliferation with strong S100 and SOX10 positivity, confirming the diagnosis of cutaneous malignant peripheral nerve sheath tumors (C-MPNST). The tumor was excised, and adjuvant radiotherapy at a dose of 64 Gy. Our findings provide valuable insights into the clinical and pathological characteristics, management strategies, and prognostic factors of C-MPNSTs.