A Case of Malignant Peripheral Sheath Tumor Arising from Neurofibromatosis Type 1
Annals of Dermatology
;
: 32-36, 2008.
Article
in English
| WPRIM
| ID: wpr-187387
ABSTRACT
Malignant peripheral nerve sheath tumor (MPNST) is a term encompassing tumors previously diagnosed as malignant schwannoma, malignant neurilemmoma, neurogenic sarcoma, and neurofibrosarcoma The occurrence rate of MPNST in neurofibromatosis type 1 patients is known to be about 4.6%. Tumors occurring in this particular group have a worse prognosis in that they occur at an earlier age, are more centrally located, tend to be of a larger size and show more metastases and recurrences. We present a typical case of MPNST in a 36-year-old man with NF type 1, which occurred on the left buttock. A PET-CT showed findings of possible inguinal lymph node metastasis and a lymph node biopsy confirmed the diagnosis. The patient was treated with wide surgical resection and is undergoing adjuvant radiation therapy.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Peripheral Nerves
/
Prognosis
/
Recurrence
/
Biopsy
/
Buttocks
/
Neurofibromatosis 1
/
Neurofibromatoses
/
Neurofibrosarcoma
/
Nerve Sheath Neoplasms
/
Lymph Nodes
Type of study:
Prognostic study
Limits:
Adult
/
Humans
Language:
English
Journal:
Annals of Dermatology
Year:
2008
Type:
Article
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