Malignant Peripheral Nerve Sheath Tumour Manifested as a Perineal Mass: A Case Study and Review Of the Literature

• John Adi Ashindoitiang

  Department of Surgery, University of Calabar Teaching Hospital, Calabar, Nigeria

• Victor Ikechukwu Canice Nwagbara

  Department of Surgery, University of Calabar Teaching Hospital, Calabar, Nigeria

• Theophilus Ipeh Ugbem

  Department of Pathology, University of Calabar Teaching Hospital, Calabar, Nigeria

• Inongha Ndoma Lifu

  Department of Pathology, University of Calabar Teaching Hospital, Calabar, Nigeria

• Effiom Umoh Okon

  Department of Pathology, University of Calabar Teaching Hospital, Calabar, Nigeria

• Maurice Efana Asuquo

  Department of Surgery, University of Calabar Teaching Hospital, Calabar, Nigeria

Background: Malignant mesenchymal tumours originating from peripheral nerves or nerve sheath cells are classified as malignant peripheral nerve sheath tumours (MPNSTs), previously referred to as neurofibrosarcoma. Most cases of these lesions occur in patients diagnosed with neurofibromatosis type 1, while some instances may be linked to previous radiation exposure, and others arise sporadically.

Case report: This report presents a 53-year-old male patient who experienced swelling in the scrotal area over the past 18 months. Clinical assessment and histopathological examination revealed a diagnosis of a perineal MPNST.

Conclusion: Perineal MPNST is infrequent, presenting distinct diagnostic challenges and should therefore be considered in the differential diagnosis of perineal masses. The diagnosis was based solely on histological examination, lacking confirmation through immunohistochemical methods, which constitutes a limitation. Surgical resection yielded positive outcomes, as there were no signs of recurrence observed three months post-surgery with a longer follow-up period deemed necessary.

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