대한흉부영상의학회 Weekly Chest Cases

Courtesy of Ki Nam Kim, MD, Ki-Nam Lee, MD. / Dong-A University Hospital, Busan, Korea
Age/Sex 27 / M
Chief ComplaintAggravation of cough for 3 months

Diagnosis: Malignant Peripheral Nerve Sheath Tumor and Multiple Neurogenic Tumors in Neurofibromatosis Type 1
Radiologic Findings: Chest radiograph showed multiple mediastinal and pleural nodules or masses. Contrast-enhanced CT scans show multiple, well-defined mediastinal and pleural masses with areas of low attenuation, and plexiform mass in right supraclavicular fossa. CT scans after 1month showed increased size of right posterior mediastinal mass with tracheal narrowing. MPNST with plexiform neurofibroma was confirmed by operation of right mediastinal mass. .

He had multiple caf?au-lait spots and cutaneous neurofibromas in his trunk. Despite of chemotherapy after operation, follow up CT revealed pulmonary metastases in both lung.
Brief Review: The most prominent manifestations of neurofibromatosis (von Recklinghausen뭩 disesase) are cutaneous caf?au-lait spots and neurofibromas of the cutaneous and subcutaneous peripheral nerves, nerve roots, and viscera. Thoracic neoplasmms are usually neurogenic. They can arise in the intercostals nerves, mediastinum, and in the lungs themselves. The most common pulmonary manifestations consists of diffuse interstitial fibrosis and bullae, either alone or in combination. The prevalence of interstitial fibrosis is about 5% - 10%, bullae in almost 20% of patients in some series.
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Keywords: Chest wall, Multiple organ, Congenital, Malignant tumor, benign tumor,

대한흉부영상의학회 Korean Society of Thoracic Radiology