CASE-REPORT - CLASSIC ADAMANTINOMA IN A 3-YEAR-OLD

Classic adamantinoma of the long bones is a rare, low-grade malignant neoplasm arising most often in the tibia and usually in patients durin g the second to fifth decades. Although adamantinomas have been descri bed in children, the histologic pattern in this age group is different from that seen in adults and resembles osteofibrous dysplasia. The us ual pattern of adamantinoma in children has been termed ''differentiat ed adamantinoma'' and follows a benign course. We report a case of ada mantinoma in the proximal tibia of a 3-year-old patient. The lesion ha d abundant epithelial component with formation of keratin pearls, a pa ttern that has: been described only in classic adamantinoma occurring in adults. Since differentiated adamantinomas are essentialy benign an d classic adamantinomas are low-grade malignancies, the finding of a c lassic variant at this young age raised important therapeutic and prog nostic issues.