Malignant peripheral nerve sheath tumor of the nose with coincidental HIV infection: Management challenges in limited resource setting

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon soft-tissue sarcoma, with origin from the peripheral nerves or somatic tissues associated with nerve sheath. This tumor rarely affects the nose. The clinicopathological presentations vary from one case to another and depend on cells of origin. Hence, the diagnosis is often difficult. We report a 36-year-old woman who presented in our facility with progressive nasal swelling, facial deformity, and mucopurulent rhinorrhea of 3 years duration. Examination revealed a significant facial disfigurement. She was also found to be seropositive. Computerized tomography scan of paranasal sinuses showed an expansile, subtle enhancing, predominantly low-density mass, with area of air density within it, completely occupying the nasal cavity. No calcific focus, lytic or sclerotic surrounding bones changes seen. Her tumor was resected under general anesthesia with a wide margin, and regional vital structures were preserved. The histological/immunohistochemistry revealed MPNST. She had adjuvant radiotherapy and chemotherapy 2 weeks after surgery. However, a nodule was seen on the floor of the nose few days after completion of radiotherapy, which was biopsied, and histological report was positive for malignancy. The persistence of the malignancy and HIV hampered her facial reconstruction. Also, limited finance hampered procurement of the nasal prosthesis. She was followed up for 1 year before she was lost.