THORACIC TUMORS IN CHILDREN WITH NEUROFIBROMATOSIS-1

Thoracic tumors have been infrequently reported as a complication of n eurofibromatosis-1 (NF1), To determine the prevalence and clinical fea tures of thoracic tumors seen in children with NF1, we reviewed medica l records and imaging studies for a group of 260 pediatric patients wi th NF1 followed in a multidisciplinary NF Center, Extrapleural thoraci c tumors were seen in nine patients with NF1, corresponding to a preva lence of 3.5% in this hospital-based series of patients, Pathological studies of the tumors demonstrated plexiform neurofibroma in four case s and neurofibrosarcoma in one case, The remaining four cases were sus pected to be plexiform neurofibroma based on clinical features but hav e not been confirmed histologically, Three patients presented with sym ptoms of chest pain, syncope, or wheezing; six patients were asymptoma tic at the time of diagnosis of the tumors, Physical findings frequent ly found in patients with thoracic tumors were scoliosis (especially f ocal scoliosis) and visible plexiform neurofibromas of the neck, We co nclude that NF1 patients presenting with any of these signs and sympto ms should be screened for thoracic tumors with chest X-ray and magneti c resonance imaging as needed, It is unknown whether screening asympto matic NF1 patients with chest X-rays on a regular basis will result in an improved outcome. (C) 1997 Wiley-Liss, Inc.