A Clinicopathological Study of Malignant Peripheral Nerve Sheath Tumors- At a Tertiary Care Center

Abstract

Aim: To study the clinicopathological profile in Malignant Peripheral Nerve Sheath Tumors (MPNST). Study Design:  A retrospective descriptive study. Place and Duration of Study: Department of Pathology, Saveetha medical college and hospital, Chennai, between June 2016 and May 2021. Methodology: A complete enumerative sampling of all the MPNSTs received at Saveetha Medical College and Hospital over a five-year period was done which included 9 cases (4 men, 5women; age range (19-66 years). The details were obtained from the records in the Department of Pathology. The demographic parameters, clinical and histomorphological data were tabulated and analysed. Results: Nine cases of MPNSTs were received in our hospital during the study period.  In our study a female preponderance was noted, with late thirties being the median age of presentation. Extremities and trunk were the most common sites. Two rare lesions involving the pancreas and mesentery were reported. 4 cases had underlying Neurofibromatosis (NF1). The average tumour size was 8.8cm, with majority having a tumour size >5cm. Histomorphologically most cases showed hypo and hypercellular areas composed of spindle cells with wavy nuclei arranged in interlacing fascicles with increased mitotic activity and tumour necrosis. Two cases showed epithelioid differentiation and three had heterologous elements in the form of glandular differentiation or rhabdomyoblastic differentiation. Conclusion: MPNSTs are very rare tumours accounting for 6% of soft tissue sarcomas and have a very aggressive course. Details of the clinical presentation and careful histomorphological typing of these tumours are essential for identifying and treating them.