Musculoskeletal ImagingMaffucci's syndrome
(Angelo Maffucci, 1847 - 1903, Italian physician), a congenital, nonhereditary mesodermal dysplasia with enchondromatosis and haemangiomas. In some cases malignant transformation takes place, usually to chondrosarcoma. The lesions may be unilateral or bilateral; they tend to occur in the metacarpals and phalanges of the hand. Lack of bone modeling may lead to limb length discrepancies and tubulation deformities. Radiographically the intraosseous lesions are central or eccentric, are radiolucent, and contain variable amounts of calcification (Fig.1). Phleboliths may be seen in the soft tissue haemangiomas.
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PA radiograph demonstates numerous phleboliths in soft tissue haemangiomas and osseous deformities owing to the presence of multipole enchondromas. An enchondroma in the index finger has undergone malignant degeneration into a chondrosarcoma; the middle finger was amputated previously owing to chondrosarcoma.
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Maffucci's syndrome, Fig.1 | |