Piriform Sinus Neurofibroma in a Patient with Neurofibromatosis Type 1: A Case Report

Abstract

Background and Objective: Neurofibromatosis is a medical disorder associated with nerve and skin tumors. These tumors can affect the brain, spinal cord and peripheral nerves. The present study was conducted to introduce a rare case of piriform sinus neurofibroma in a patient with neurofibromatosis type 1. Case Report: The patient is an 11-year-old boy who had a history of surgery for neurofibroma on the right side of the neck and was referred for mass removal surgery due to a palpable mass on the left side of the neck, with no other specific symptoms. During the preparation for the surgery, the anesthesiologist noticed a mass in the larynx area, which led to surgery cancellation and the MRI images were reviewed again. After two weeks, the patient returned to the hospital to have the laryngeal mass removed. During laryngoscopy, the surgeon identified and removed a submucosal mass in the larynx area. After examination of the lesion by a pathologist, asymptomatic neurofibroma was diagnosed. The primary treatment for this patient included surgery to remove the neurofibromatosis-associated tumors. The result of the treatment was successful, and a one-year follow-up treatment was carried out, which included post-surgery care, periodical imaging, evaluation of the nervous system and examination of other symptoms of neurofibromatosis along with close monitoring. Conclusion: Based on the results of this study, the identification of neurofibromatosis-associated masses in uncommon places such as the larynx requires precision in imaging and surgical evaluations. © 2024 Babol University of Medical Sciences. Published by Babol University of Medical Sciences.