Giant cell fibroblastoma is a rare mesenchymal neoplasm of unknown ori
gin and uncertain clinical course. The neoplasm has been considered by
some authors as a juvenile variant of dermatofibrosarcoma protuberans
. We report a patient with giant cell fibroblastoma, now 28 months fol
lowing surgical removal, in which the neoplasm was characterized histo
logically by a proliferation of spindle-shaped cells intermixed with p
seudovascular channels called ''angiectoid spaces.'' The spaces were l
ined by large cells with pleomorphic nuclei intermixed with multinucle
ated cells. Immunohistochemically, the tumor stained diffusely for vim
entin and CD34, a surface glycoprotein expressed by some mesenchymal n
eoplasms including dermatofibrosarcoma protuberans. We postulate a mec
hanism of formation of the angiectoid spaces based on histopathologica
l findings in serially sectioned portions of the neoplasm. Positive st
aining of tumor cells for CD34 supports a possible relationship of the
neoplasms with dermatofibrosarcoma protuberans.