Korean J Med.
2009 Feb;76(2):234-237.
An intrathoracic malignant peripheral nerve sheath tumor in a neurofibromatosis type 1 patient
- Affiliations
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- 1Department of Internal Medicine, Kwandong University College of Medicine, Myongji Hospital, Koyang, Korea. pulmochung@kwandong.ac.kr
- 2Department of Pathology, Kwandong University College of Medicine, Myongji Hospital, Koyang, Korea.
- 3Department of Radiology, Kwandong University College of Medicine, Myongji Hospital, Koyang, Korea.
Abstract
- A malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue tumor defined as any malignant tumor arising from or differentiating toward the cells of the peripheral nerve sheath. It is one of the malignant tumors associated with neurofibromatosis type 1 (von Recklinghausen's disease). They occur most commonly on the lower and upper extremities, trunk, head, and neck, while intrathoracic tumors are very rare. We report an intrathoracic MPNST in a 66-year-old female with neurofibromatosis type 1.
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