SYMMETRICAL ENCHONDROMATOSIS OF THE HANDS AND FEET IN 2 SISTERS

We report two Portuguese sisters aged 9 and 12 years with symmetric we ll-circumscribed radiolucent cystic lesions on the long bone metaphysi s of the hands and feet. The eldest also has soft tissue calcification s. They have no dysmorphic features and their growth is normal. Plasma values of parathyroid hormone (PTH), calcium, phosphorus, magnesium, and alkaline phosphatase are normal. Cerebral computed tomography (CT) scan shows no intracranial calcifications. A Raynaud phenomenon becam e evident during the last year in the eldest. The incisional biopsy of the left proximal metatarsial was performed through an area of typica l radiographic appearance. The pathology specimen consisted of enchond roma tissue. The present cases are an extremely rare instance of this pathology, with symmetrical involvement of the hands and feet and a fa milial incidence.