Neurofibromatosis type 1: evaluation by chest computed tomography

Abstract Objective: The aim of this study was to evaluate chest computed tomography (CT) findings in patients diagnosed with neurofibromatosis type 1 (NF1). Material and Methods: This was a retrospective study in which we reviewed the chest CT scans of 14 patients diagnosed with NF1 and neurofibromatosis-associated diffuse lung disease (NF-DLD). The sample comprised eight women and six men. The median age was 55 years (range, 11-75 years). The diagnosis of NF1 was made on the basis of the diagnostic criteria established by the U.S. National Institutes of Health. The images were analyzed by two chest radiologists, who reached decisions by consensus. Results: The predominant CT finding of NF-DLD was multiple cysts, which were observed in 13 patients (92.9%), followed by emphysema, in eight (57.1%) and subpleural bullae, in six (42.9%). Other findings included subcutaneous neurofibromas, in 12 patients (85.7%), ground-glass opacities, in one (7.1%), and tracheobronchial neurofibromas, in one (7.1%). The pulmonary abnormalities were bilateral in 12 cases (85.7%). The abnormalities were predominantly in the upper lung fields in eight cases (57.1%), and their distribution was random in 11 (78.6%). Conclusion: Pulmonary cysts, emphysema, and subpleural bullae appear to be the chest CT findings that are most characteristic of NF-DLD.

Bibliographic Details

Main Authors:	Alves Júnior,Sérgio Ferreira, Irion,Klaus Loureiro, Melo,Alessandro Severo Alves de, Meirelles,Gustavo de Souza Portes, Rodrigues,Rosana Souza, Souza Jr.,Arthur Soares, Hochhegger,Bruno, Zanetti,Gláucia, Marchiori,Edson
Format:	Digital revista
Language:	English
Published:	Publicação do Colégio Brasileiro de Radiologia e Diagnóstico por Imagem 2021
Online Access:	http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0100-39842021000600375
Tags:	Add Tag No Tags, Be the first to tag this record!