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Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans
Author(s) -
Shmookler Barry M.,
Enzinger Franz M.,
Weiss Sharon W.
Publication year - 1989
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19891115)64:10<2154::aid-cncr2820641030>3.0.co;2-n
Subject(s) - dermatofibrosarcoma protuberans , histogenesis , dermatofibrosarcoma , giant cell , medicine , pathology , metastasis , sarcoma , lesion , juvenile , biology , cancer , immunohistochemistry , genetics
A clinicopathologic analysis of 28 cases of giant cell fibroblastoma (GCF), a rare mesenchymal tumor occurring predominantly in the first decade of life, is presented. This disease presented as a painless, slowly enlarging, subcutaneous mass. The tumor recurred locally in 47% of the patients; however, metastasis was not detected. On microscopic examination, GCF showed an unique combination of spindle cell patterns, pleomorphic and multinucleated giant cells, myxoid areas, and distinctive sinusoid‐like spaces. This unrecognized histomorphologic picture led to a misdiagnosis of sarcoma in 40% of the cases. The histogenesis of this lesion remains uncertain; however, based on both clinical and morphologic similarities, it is proposed that GCF is a juvenile form of dermatofibrosarcoma protuberans (DFSP).