Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I: Unusual Presentation of Intraabdominal or Intrathoracic Mass.
10.3904/kjim.2005.20.1.100
- Author:
Jong Gwang KIM
1
;
Woo Jin SUNG
;
Dong Hwan KIM
;
Young Hwan KIM
;
Sang Kyun SOHN
;
Kyu Bo LEE
Author Information
1. Department of Oncology/Hematology, Kyungpook National University Hospital, Daegu, Korea. sksohn@knu.ac.kr
- Publication Type:Case Report
- Keywords:
Neurofibromatosis;
Sarcoma;
Chemotherapy
- MeSH:
Abdominal Neoplasms/*diagnosis;
Adult;
Female;
Humans;
Nerve Sheath Tumors/*diagnosis;
Neurofibromatosis 1/*complications;
Thoracic Neoplasms/*diagnosis
- From:The Korean Journal of Internal Medicine
2005;20(1):100-104
- CountryRepublic of Korea
- Language:English
-
Abstract:
A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population. On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease). The common sites are the extremities, trunk, head and neck. However, an intraabdominal or intrathoracic manifestation is uncommon. This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor. The patients were treated with chemotherapy followed by radiotherapy. However, one patient died as a result of disease progression 21 months after the diagnosis and the other patient is currently being treated with radiotherapy.
