Journal List > J Korean Soc Radiol > v.63(4) > 1086839

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Shin, Lee, Moon, and Sung: Neurofibromatosis Type I presenting with Spontaneous Pneumothorax: A Case Report
Chest Radiology
Case Report

Journal of the Korean Society of Radiology 2010; 63(4): 379-382.

Published online: 31 October 2010

DOI: https://doi.org/10.3348/jksr.2010.63.4.379

Neurofibromatosis Type I presenting with Spontaneous Pneumothorax: A Case Report

So Youn Shin, M.D., Young Kyung Lee, M.D., Ah Lim Moon, M.D.2, Dong Wook Sung, M.D.3

Department of Radiology, East-West Neo Medical Center, Kyung Hee University, Korea.

2Department of Pathology, East-West Neo Medical Center, Kyung Hee University, Korea.

3Department of Radiology, Kyung Hee University Hospital, Kyung Hee University, Korea.

Address reprint requests to: Young Kyung Lee, M.D., Department of Radiology, East-West Neo Medical Center, Kyung Hee University, 149, Sangil-dong, Gangdong-gu, Seoul 134-727, Korea. Tel. 82-2-440-6933, Fax. 82-2-440-6932, ykradio@medimail.co.kr

Received 7 May 2010       Accepted 29 July 2010

Copyright © 2010 The Korean Society of Radiology

Abstract

Neurofibromatosis type I is an autosomal dominant disease with variable clinical manifestations related to dermatologic, neurologic, skeletal, and endocrine system. Lung parenchymal involvement such as lung fibrosis and massive bullous emphysema is infrequent. Here, we report on a 36-year-old man with symptoms of dyspnea, and who has a spontaneous pneumothorax, multiple bullae, and pathologically confirmed neurofibromatosis type I.

Keywords: Pneumothorax, Blister, Neurofibromatosis

Figures and Tables

Fig. 1

A. Chest radiograph shows the large, well-demarcated lucent area devoid of lung markings in left hemithorax without tracheal deviation or mediastinal shifting and with the visceral pleural line (arrows). Note the large thin-walled cyst in right upper lung zone (asterisk).

B-D. Axial chest CT scans show multiple thin-walled bullae with predominant distribution in the both upper lobes (B, C). Axial chest CT scan shows multiple thin-walled cysts in both lower lobes and atelectasis in left lower lobe. Small left pneumothorax (arrow) and left tube thoracostomy are noted (D).
E. The microscopic examination shows tumor cells with a wavy, spindle appearance in a myxoid matrix (H & E stain, × 100).
jksr-63-379-g001

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