Synchronous intracortical adamantinomas with keratin cyst formation

Adamantinoma of the long bones is a rare primary bone tumor of uncertain embryogenesis. It tends to involve the tibia almost exclusively. We report on adamantinomas occurring in a 16-year-old male patient, with synchronous tibial and fibular lesions. Histologically, there were characteristic clusters of epithelial cells in a fibrous background, forming a keratin cyst. Immunohistochemically, these cells were strongly positive for cytokeratin. This keratin cyst formation is quite an unusual finding in classic adamantinoma.