Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans.
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).
Shmookler
B M
BM
Department of Pathology, Washington Hospital Center, Washington, DC 20010.
Enzinger
F M
FM
Weiss
S W
SW
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003094:Collagen / Q000032:analysis; D004721:Endoplasmic Reticulum / Q000648:ultrastructure; D005260:Female; D005354:Fibrosarcoma / Q000473:pathology* / Q000601:surgery / Q000648:ultrastructure; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D012878:Skin Neoplasms / Q000473:pathology* / Q000601:surgery / Q000648:ultrastructure
D003094:Collagen