Malignant Fibrous Histiocytoma with Type I Neurofibromatosis.
- Author:
Se Ho PARK
1
;
Kang Young LEE
;
Nam Kyu KIM
;
Seung Kook SOHN
;
Nae Choon YOO
;
Hyo Sup SHIM
;
Se Hoon KIM
;
Tai Seung KIM
;
Chang Hwan CHO
Author Information
1. Department of Surgery, Yonsei University College of Medicine, Seoul, Korea. chcho@yumc.yonsei.ac.kr
- Publication Type:Case Report
- Keywords:
Neurofibromatosis;
Malignant fibrous histiocytoma
- MeSH:
Adult;
Cafe-au-Lait Spots;
Female;
Histiocytoma, Malignant Fibrous*;
Humans;
Neural Crest;
Neurofibroma;
Neurofibromatoses*;
Neurofibromatosis 1;
Parturition;
Sarcoma;
Skin
- From:Journal of the Korean Surgical Society
2004;67(2):167-170
- CountryRepublic of Korea
- Language:Korean
-
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.
