A Giant Malignant Peripheral Nerve Sheath Tumour: A Report of Rare Case

Kefas Mari Mbaya; Mujtaba Bala; Abba Bukar Zaramai; Audi Innuwa Kwari; Salihu Aliyu; Umar  Abdullahi

doi:10.5455/CRSM.20250326010836

Abstract
Malignant peripheral nerve sheath tumor is a rare malignant neoplasm of the peripheral nerve sheath and can occur anywhere in the body. The most common location is the deep tissue of the arms, legs, and trunk; however, it is extremely rare in the head and neck region. We report a case of a 9-year-old girl who presented with a 3-year history of swelling in the occipital region. The swelling was said to have started as a small nodule which gradually progressed to form a large mass. It was initially painless but became painful at about 6 months before presentation. The swelling is single, firm, non-tender, and has no differential warmth. Thorough history taking and careful examination are vital to the diagnosis of this tumor. The diagnosis was confirmed via histopathological examination and she had surgical excision of the tumor under general anesthesia with excellent outcome on follow-up.

Key words: Peripheral nerve sheath, tumor, occipital region, surgery, chemotherapy, radiotherapy