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
in English
| 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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Thoracic Neoplasms
/
Neurofibromatosis 1
/
Nerve Sheath Neoplasms
/
Abdominal Neoplasms
Limits:
Adult
/
Female
/
Humans
Language:
English
Journal:
The Korean Journal of Internal Medicine
Year:
2005
Type:
Article
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