DOI: 10.36347/sjams.2020.v08i08.015

Pages: 1871-1873

Neurofibromatosis type 1 (NF1) or von Recklinghausen’s disease is a phakomatosis transmitted by autosomal dominant inheritance. It is diagnosed when some criteria are present in the same individual. Patients with NF1 tend to develop neurofibromas which is a are benign tumor of peripheral nerve sheath cells. We describe the rare case of a 32-year-old man with NF1 and a bilateral plexiform neurofibroma of the sciatic nerve. Given the high surgical risk, the patient was maintained under clinical and radiological surveillance. The evolution is unpredictable and current treatment options are limited to surgical resection. Radiation is not used because it could actually promote malignant transformation and it’s known that neurofibroma is non-responsive to chemotherapy. Angiotensin-converting enzyme inhibitors have been proposed as a novel treatment of neurofibromas and recently the administration of interferon- gave good results in unresectable, progressive and symptomatic lesions.