Department of Pathology, University of Pretoria, Pretoria, South Africa
Case Report
Plexiform Neurofibroma Transformation to a Malignant Peripheral Nerve Sheath Tumour: A Case Report in a Patient with Neurofibromatosis-1
Author(s): Tebogo Linky Medupe*
One of the most prevalent dominantly inherited genetic disorders is neurofibromatosis-1. The majority of epidemiological research has indicated that at least one in three thousand people experience this globally. Neurofibromatosis-1 people are more likely to develop cancer, and the illness affects several systems, with primary manifestations occurring in the cutaneous, neurologic, and orthopaedic sites. These conditions can cause severe morbidity or fatality. The neurocutaneous-skeletal Neurofibromatosis-1 (NF1) syndrome can only be caused by a mutation in the NF1 gene; nevertheless, the pathogenesis of the disease's various manifestations in various organ systems appears to be becoming more complex. The formation, severity, and prognosis of a wide range of distinct clinical phenotypes appear to be caused by interactions between many cell types, cell signaling net.. View more»