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KMID : 0613319990050020144
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Journal of the Korean Bone and Joint Tumor Soceity
1999 Volume.5 No. 2 p.144 ~ p.149
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Atypical Maffucci’s Syndrome Associated with Arteriovenous Malformation and Cafe-au-Lait Spot -A Case Report-
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Park Chan-Sik
Kim Jong-Jae
Jang Ja-June
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Abstract
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Maffucci’s syndrome is a rare, nonhereditary, congenital disorder consisting of enchondromatosis in association with hemangiomas. It is frequently associated with dysplasia of mesodermal tissues and various tumors. We report a case o# atypical Maffucci’s syndrome which clinically mimicked features of Mccune-Albright syndrome due to the association of complex lesion. A 23-year-old male patient was transferred for surgical correction of non-union of a right humeral fracture. Ten years prior to this admission, he initially presented with a multiple arteriovenous malformations and multiple osteolytic bony lesions predominantly affecting the right side of the extremities, cafe-au-lait spots on the back and groin, and radiologically-suspected pituitary adenoma. This time, above-elbow amputation was done because of massive bleeding from arteriovenous malformations at the fracture site. Large hematoma containing large vessels occupied the fracture site of the humerus shaft and multiple enchondromas were found incidentally in distal humerus, ulnar, radius, and digital bones. Microscopic examination showed multiple enchondromas with minimal atypia, many hi-nucleated cells within a lacuna, and arteriovenous malformations within the marrow space and adjacent soft tissue. The clinicopathologic profile of this case is quite unusual for Maffucci’s syndrome, and has not previously been reported in the literature.
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KEYWORD
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Maffucci’s syndrome, Enchondromatosis, Fibrous dysplasia, Arteriovenous malformation, Cafe-au-lait spot, Pituitary adenoma
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