title: Intrathoracic malignant peripheral nerve sheath tumor in von Recklinghausen's disease.
abstract: Malignant peripheral nerve sheath tumor (MPNST) is defined as any malignant tumor arising from or differentiating toward the cells of the peripheral nerve sheath. MPNST accounts for about 5-10% of all soft tissue tumors and is often associated with neurofibromatosis type I (NF-1, von Recklinghausen's disease). It is one of the malignant tumors associated with von Recklinghausen's disease. Its common site is the lower and upper extremities, trunk, head and neck. But intrathoracic manifestations are very rare. We report a case of a 40 year-old man with multiple neurofibromatosis who was presented with an intrathoracic malignant peripheral nerve sheath tumor.
authors.affiliation: Department of Internal Medicine, Chonbuk National University, School of Medicine, 634-18 Keumam-dong, Duckjin-ku, Chonju 561-712, Chonbuk, South Korea.
authors.forename: J H
authors.initials: JH
authors.lastname: Park
authors.forename: K H
authors.initials: KH
authors.lastname: Choi
authors.forename: H B
authors.initials: HB
authors.lastname: Lee
authors.forename: Y K
authors.initials: YK
authors.lastname: Rhee
authors.forename: Y C
authors.initials: YC
authors.lastname: Lee
authors.forename: M J
authors.initials: MJ
authors.lastname: Chung
mesh_terms: D000328:Adult; D003964:Diaphragm / Q000473:pathology; D006801:Humans; D008168:Lung / Q000473:pathology; D008297:Male; D018317:Nerve Sheath Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D011379:Prognosis; D012720:Severity of Illness Index; D013899:Thoracic Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology*