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KMID : 0371320040670020167
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Journal of the Korean Surgical Society
2004 Volume.67 No. 2 p.167 ~ p.170
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Malignant Fibrous Histiocytoma with Type I Neurofibromatosis
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Park Se-Ho
Rhee Kang-Young
Kim Nam-Kyu
Sohn Seung-Kook
Yoo Nae-Choon
Shim Hyo-Sup
Kim Se-Hoon
Kim Tae-Seung
Cho Jang-Hwan
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Abstract
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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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