Malignant Fibrous Histiocytoma with Type I Neurofibromatosis
Journal of the Korean Surgical Society
;
: 167-170, 2004.
Article
in Korean
| WPRIM
| ID: wpr-92214
ABSTRACT
Type I neurofibromatosis is an autosomal dominant disorder that occurs in 1 of 3, 000 births. It is characterized by multiple cutaneous neurofibromas, cafe-au-lait spots of the skin. Patients with neurofibromatosis are at increased risk of developing malignancies, particularly neural crest and other non-neural crest neoplasms. The term `malignant fibrous histiocytoma' was first introduced in 1963 to refer to a group of soft tissue tumors characterized by a storiform or cartwheel like growth pattern. Malignant fibrous histiocytomas are the most common type of soft tissue sarcoma that occurs in late adult life. Herein, our recent experienced a case of a malignant fibrous histiocytoma in a 28 year-old female with type I neurofibromatosis is reported, with a review of the literature.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Sarcoma
/
Skin
/
Neurofibromatosis 1
/
Neurofibromatoses
/
Cafe-au-Lait Spots
/
Parturition
/
Histiocytoma, Malignant Fibrous
/
Neural Crest
/
Neurofibroma
Limits:
Adult
/
Female
/
Humans
Language:
Korean
Journal:
Journal of the Korean Surgical Society
Year:
2004
Type:
Article
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