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.
Artigo
em Inglês
| WPRIM
| ID: wpr-94692
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Neoplasias Torácicas
/
Neurofibromatose 1
/
Neoplasias de Bainha Neural
/
Neoplasias Abdominais
Limite:
Adulto
/
Feminino
/
Humanos
Idioma:
Inglês
Revista:
The Korean Journal of Internal Medicine
Ano de publicação:
2005
Tipo de documento:
Artigo
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