J Korean Surg Soc.
2004 Aug;67(2):167-170.
Malignant Fibrous Histiocytoma with Type I Neurofibromatosis
- Affiliations
-
- 1Department of Surgery, Yonsei University College of Medicine, Seoul, Korea. chcho@yumc.yonsei.ac.kr
- 2Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
- 3Department of Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea.
- 4Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
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.
