Malignant Degeneration of a Peripheral Nerve Sheath Tumor in the Neck Region.

Abstract

Background: Malignant transformation of neurofibromas into malignant peripheral nerve sheath tumors (MPNST) is a rare but aggressive phenomenon, particularly in the head and neck region, and is associated with a poor prognosis. The primary treatment modality is surgical excision, often followed by radiotherapy. This is a case report of MPNST in the neck, with long-term follow-up. Case Presentation: A 26-year-old woman presented with a history of multiple progressively enlarging neck swellings for 8 years. The swellings were associated with mild pain and occasional bleeding. The patient had undergone 3 prior surgical excisions, with a fourth recurrence leading to complete excision and split-thickness skin grafting due to extensive raw surface area. Histopathological examination confirmed the diagnosis of MPNST. The patient subsequently received adjuvant chemoradiotherapy due to the aggressive nature of the pathology. Results: The patient has been under regular follow-up for 36 months, with no evidence of local, regional, or distant metastasis. Conclusion: Malignant transformation of neurofibromas is a rare entity, particularly in the head and neck. Early surgical intervention followed by adjuvant chemoradiotherapy provides effective long-term control of the disease and improved survival.