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KMID : 0613320050110020208
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Journal of the Korean Bone and Joint Tumor Soceity
2005 Volume.11 No. 2 p.208 ~ p.212
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Maffucci Syndrome Enchondromatosis of Hand with Hemangioma of Scalp A Case Report
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Chung Kyung-Chi
Kim Key-Yong
Choi Jang-Seok
Kim Jin-Seok
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Abstract
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Maffucci syndrome was first reported by Maffucci, an Italian, in 1881. This rare, nonhereditary syndrome is characterized by multiple enchondromas combined with hemangiomas and less commonly lymphangiomas. Maffucci syndrome is sometimes confused with Ollier disease, which was described in 1899 and consists of multiple enchondromas without hemangiomas. Although the prevalence of malignant change in skeletal lesions in Maffucci syndrome and Ollier disease is quite similar, it is important to differentiate between these two conditions because of the greater risk of developing nonmusculoskeletal malignancies in the former. We experienced a case of this rare syndrome and because of it’s rarity, we report with review of literature.
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KEYWORD
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Maffucci syndrome, Enchondromatosis, Hemangioma
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