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J Korean Surg Soc. 2004 Aug;67(2):167-170. Korean. Case Report.
Park SH , Lee KY , Kim NK , Sohn SK , Yoo NC , Shim HS , Kim SH , Kim TS , Cho CH .
Department of Surgery, Yonsei University College of Medicine, Seoul, Korea. chcho@yumc.yonsei.ac.kr
Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
Department of Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea.
Department 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.

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