Intrathoracic malignant peripheral nerve sheath tumor in von Recklinghausen's disease.
- Author:
Ju Hyung PARK
1
;
Koang Ho CHOI
;
Heung Bum LEE
;
Yang Keun RHEE
;
Yong Chul LEE
;
Myung Ja CHUNG
Author Information
1. Department of Internal Medicine, Chonbuk National University, School of Medicine, 634-18 Keumam-dong, Duckjin-ku, Chonju 561-712, Chonbuk, South Korea.
- Publication Type:Original Article
- Keywords:
Malignant peripheral nerve sheath tumor;
Thoracic cavity;
Von Reckinghausen's disease
- MeSH:
Adult;
Case Report;
Diaphragm/pathology;
Human;
Lung/pathology;
Male;
Nerve Sheath Tumors/complications/diagnosis/*pathology;
Neurofibromatosis 1/complications/diagnosis/*pathology;
Prognosis;
Severity of Illness Index;
Thoracic Neoplasms/complications/diagnosis/*pathology
- From:The Korean Journal of Internal Medicine
2001;16(3):201-204
- CountryRepublic of Korea
- Language:English
-
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
