Malignant Transformation of the Plexiform Neurofibroma of the Back: A Case Report

Neurofibromatosis (NF) is characterised by multiple skin lesions distributed across the body and follows an autosomal dominant inheritance pattern. There are two main subtypes: NF type 1 (NF1) and NF type 2 (NF2), each with distinct clinical features. NF1 typically presents with numerous cutaneous neurofibromas, café-au-lait spots, plexiform neurofibromas, Lisch nodules, freckling in the axillary or inguinal regions, and optic gliomas. In contrast, NF2 is marked by bilateral vestibular schwannomas and central nervous system tumours such as meningiomas and ependymomas. A 69-year-old male presented with a swelling on his back. He had a malignant tumour that had transformed from plexiform NF to neurofibrosarcoma. A wide-excision biopsy of the swelling was done and a Malignant Peripheral Nerve Sheath Tumour (MPNST) was given as a diagnosis on Histopathological Examination (HPE). He underwent surgery for excision of the swelling. The patient had undergone Contrast-Enhanced Computed Tomography (CECT) of the thorax, abdomen and pelvis, showing cystic bronchial changes in the lower lobe and minimal in the posterior segment of the right lower lobe. Also, there was evidence of a large heterogeneous enhancing lobulated soft tissue density lesion in the subcutaneous plane of the neck and upper back. NF can be prevented from progressing if the malignant change is identified early. Surgical excision is the primary therapy; nevertheless, there is a greater chance of local recurrence, particularly in those suffering from NF1. The patient had undergone wide local excision with vacuum-assisted closure, which was followed by skin grafting.