Surgery for Peripheral Nerve Sheath Tumours of the Buttocks Legs and Feet in 90 Patients With Neurofibromatosis Type 1

摘要

Background/Aim: Neurofibromatosis type 1 (NF1) is an autosomal dominant tumour predisposition syndrome that can cause plexiform neurofibromas (PNFs). This study examines the surgical procedures that have been performed on large PNFs of the lower extremities. Patients and Methods: Surgical procedures on the lower extremity performed on 90 patients with NF1 with PNFs were evaluated. The topography of the tumours was classified according to dermatomes and functional units. Results: A total of 243 surgical interventions on the regions of interest were performed. Neurological complications were rarely noted and usually occurred temporarily. There was no preference for dermatomes affected by PNF. The proportion of patients with malignant peripheral nerve sheath tumours (MPNSTs) in this group was 4/90 (4.4%). Conclusion: PNFs often require repeated local interventions to achieve the treatment goal. Local tumour recurrences are to be expected even after extensive tumour reduction. Rapid tumour growth combined with new pain sensations can be signs of a MPNST.