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Journal of the Korean Child Neurology Society 2011;19(2):165-168.
Published online August 30, 2011.
A Case of Intrathoracic Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I.
Young Mi Kim, So Eun Jeon, Byung Ki Lee, Yoon Jin Lee, Sang Ook Nam, Young Tak Lim
Department of Pediatrics, College of Medicine, Pusan National University Hospital, Busan, Korea. pink2129@naver.com
Malignant peripheral nerve sheath tumor (MPNST) is a rare neoplasm and the main cause of the mortality in neurofibromatosis type 1 (NF 1). MPNSTs have been found mostly in the head and neck and the upper or lower extremities with intrathoracic MPNSTs being uncommon. PET has been a useful diagnostic modality of MPNSTs in NF 1. We present a 17-year-old girl patient with NF 1. She was admitted with chronic cough and shortness of breath caused by a huge mediastinal mass. An 18FDG-PET study revealed intense uptake at the mediastinal mass. She underwent surgery to lessen respiratory symptoms, and the mass was histologically diagnosed as an intrathoracic MPNST.
Key Words: Malignant peripheral nerve sheath tumors, Neurofibromatosis 1, Intrathoracic


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