CASE REPORT: Spinal Malignant Peripheral Nerve Sheath Tumours in Nigerians.

West Afr J Med. 2021 Jan; 38(1): 93-97. PMID: 33463714

Abstract

Background: Spinal Malignant peripheral nerve sheath tumours (MPNSTs) are very rare aggressive tumours with poor prognosis. Little is known about these tumours in sub-saharan Africa.

Objectives: This study aims to evaluate the clinical profile and outcome of management of these tumours in a resource limited country.

Methods: We retrospectively analysed data from the records of patients who had surgery for spinal MPNSTs at our center between January 2004 and December 2018.

Results: There were four patients in this study (M:F= 1:1). The ages ranged from 27-53 years with a mean of 43.25 ± 11.84 years. The tumour was located in the thoracic region in 2 of the patients (50%), the lumbar region in one (25%) and thoracolumbar in the 4th patient. Three patients (75%) presented with back pain while limb weakness, sensory deficit and sphincteric dysfunction were present in all patients at presentation. The duration of symptoms were 2 months in 2 patients (50%) and 3 months in the other 2. None of the patients had neurofibromatosis. Gross total tumour excision was achieved in 2 patients (50%) and subtotal resection in the other 2. The tumours were high grade in three patients (75%) and low grade in one. Two patients had adjuvant radiotherapy. Two of the patients were dead within 6 months of the diagnosis, another one within 18 months while one patient is still alive 3 years after.

Conclusions: MPNSTs are very rare in our practice. Most of the tumours were high grade tumours and ran an aggressive course.