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
Dans Anglais
| 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.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Tumeurs du thorax
/
Neurofibromatose de type 1
/
Tumeurs des gaines nerveuses
/
Tumeurs de l'abdomen
Limites du sujet:
Adulte
/
Femelle
/
Humains
langue:
Anglais
Texte intégral:
The Korean Journal of Internal Medicine
Année:
2005
Type:
Article
Documents relatifs à ce sujet
MEDLINE
...
LILACS
LIS