Giant Neurofibroma of the Labia Majora, Excision With a Modelling Technique

Abstract

Background. Neurofibromatosis type 1 (NF1) is an autosomal dominantly inherited neurocutaneous disease caused by a mutation in the neurofibromin gene on chromosome 17q11.22. NF1 is a multisystem disease, and patients with this condition are at an increased risk of developing both benign and malignant tumors of the central and peripheral nervous systems. Although rare, NF can affect the genital tract, with the vulva being the most common site and involvement of the vagina, cervix, and ovaries being reported less frequently. Genital involvement often causes pain and psychological discomfort for patients, and surgical removal is the mainstay of treatment due to the large innervation of the area. Material and methods. This work describes the case of a 41-year-old patient with a known diagnosis of NF1 who presented with a large painful mass in the left labium majus. The mass was surgically treated with a modeling excision technique. Results. At the six-month follow-up visit, the patient reported being very satisfied with the results, and no complications were observed. Conclusions. Conservative modeling surgical excision appears to be the most appropriate technique for managing giant genital NF. This technique minimizes the risk of bleeding, restores correct anatomy and sexual function, and has a positive impact on the patient's psychological well-being.