ADAMANTINOMA OF LONG BONES - A HISTOPATHOLOGICAL AND IMMUNOHISTOCHEMICAL STUDY OF 23 CASES

The clinical and histological data of twenty-three cases of adamantino mas of the long bones collected by the Working Group on Bone Tumors at the DKFZ/FRG are reported including immunohistochemical observations in twenty-one of the cases. Females and males between 5 and 67 years ( mean, 25.4 years) were affected equally (11/12). All adamantinomas wer e positive for cytokeratins often in coexpression with vimentin, at le ast focally. Although exhibiting varying histological patterns, no cor relation between histology and clinical course was seen. However, sex and mode of initial therapy seem to influence an unfavorable clinical outcome. All three deceased patients were males receiving marginal or delayed surgery. This underlines the low-grade malignant character of adamantinoma. To assure the histological diagnosis pathologists should employ immunohistochemistry for demonstrating the sometimes sparse ep ithelial cell nests when radiology is suggestive for adamantinoma. Cor rect diagnosis should lead to resection with wide surgical margins.