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KMID : 0391520110190020165
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Journal of the Korean Child Neurology Society
2011 Volume.19 No. 2 p.165 ~ p.168
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A Case of Intrathoracic Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I.
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Kim Young-Mi
Jeon So-Eun
Lee Byung-Ki
Lee Yoon-Jin
Nam Sang-Ook
Lim Young-Tak
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Abstract
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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.
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KEYWORD
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Malignant peripheral nerve sheath tumors, Neurofibromatosis 1, Intrathoracic
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