Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I: Unusual Presentation of Intraabdominal or Intrathoracic Mass
The Korean Journal of Internal Medicine
; : 100-104, 2005.
Article
em En
| WPRIM
| ID: wpr-94692
Biblioteca responsável:
WPRO
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.
Palavras-chave
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Neoplasias Torácicas
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Neurofibromatose 1
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Neoplasias de Bainha Neural
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Neoplasias Abdominais
Limite:
Adult
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Female
/
Humans
Idioma:
En
Revista:
The Korean Journal of Internal Medicine
Ano de publicação:
2005
Tipo de documento:
Article