By Gary M. White, MD
Maffucci syndrome represents the combination of unilateral vascular lesions with encondromas most commonly occurring on the hand and fingers.
- The vascular lesions include vascular malformations (venous or lymphatic), telangiectasias, and phlebectasias.
- The venous malformations are often present at birth, do not enlarge out of proportion to body size and do not regress. They may also present in childhood.
- Enchondromas are tumors where persistence of cartilage expands within the bone and interferes with normal bony growth at the epiphyseal plate.
- Deformity or shortening of the affected limb may occur. The metacarpals and phalanges are most frequently affected.
- Malignant degeneration of an enchondroma to a chondrosarcoma can occur.
- Bone pain and enlargement may be signs of malignant change.
- Other malignant mesenchymal tumors may occur.
Referral to a specialist is in order.
An 18-year-old girl ppresents with multiple soft tissue and bony painless swellings over fingers, toes and ankles since the age of 3½ years. Indian Pediatrics
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