Surgical treatment of a patient with giant perineal neurofibroma

S.A. Bystrov, E.V. Shestakov, A.I. Bezborodov, O.E. Lisin, A.V. Arustamyan, E.V. Semenaya, S.V. Poletaeva, S.E. Katorkin

Samara State Medical University, Samara

The article describes the successful surgical treatment of a patient with neurofibromatosis type I and giant perineal neurofibroma, which hinder movements and interfere with hygiene procedures. The 60x30 cm tumor spread to the right gluteal and perianal areas, the right labia majora area, and the posterior surface of the right thigh, significantly deteriorating the patient’s life quality. The choice of surgical treatment tactics was determined by the huge tumor size. Intraoperatively, special attention was paid to perianal and perineal areas, where local flaps grafting was used to hide the defect. When removing abnormal tissues, it was necessary to avoid damage to the sphincter muscle fibers and thereby prevent the development of fecal incontinence. Blood components (fresh frozen plasma, red blood cell suspension) were transfused intraoperatively. Blood loss was 300 ml. In the early postoperative period (2 months  after the operation), a satisfactory condition of post-operative scars and a significant improvement in the life quality were noted. Healing by primary intention. Anal sphincter function is not impaired. The patient performs hygiene procedures without any difficulties.

Keywords: von Recklinghausen’s disease, peripheral neurofibromatosis, phacomatosis, myxoid neurofibroma, surgical treatment.

For citation: Bystrov S.A., Shestakov E.V., Bezborodov A.I. et al. Surgical treatment of a patient with giant perineal neurofibroma. RMJ. 2020;13:45–47.