Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans

Abstract

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).

Benign tumors of the breast with multinucleated stromal giant cells

“…However, we consider this to be highly significant and constant feature, as it was observed in all of our cases and is also visible in some figures in previous reports [16,19]. In some areas the stroma strongly resembled the appearance of giant cell fibroblastoma (GCF), where a pseudoangiomatous pattern is a feature [12,23]. Another lesion featuring spaces reminiscent of vascular lumina is pseudoangiomatous stromal hyperplasia of the mammary gland (PASH) [2,20,26].…”

Orbital solitary fibrous tumor: encompassing terminology for hemangiopericytoma, giant cell angiofibroma, and fibrous histiocytoma of the orbit: reappraisal of 41 cases

“…We independently and a priori observed that several tumors had angiectatic spaces lined by floret-type giant cells and stromal collagen, identical to those observed in a known soft tissue tumor, GCF. GCF is primarily a truncal tumor of childhood, the myxoid, benign variant of adult dermatofibrosarcoma protuberans (DFSP) [39,40]. Although there has been a case associating DFSP with GCA in the skin [41], we suspect rather that this case was probably a combination of GCF and DFSP, a common hybrid occurrence.…”

Dermatofibrosarcoma protuberans COL1A1-PDGFB fusion is identified in virtually all dermatofibrosarcoma protuberans cases when investigated by newly developed multiplex reverse transcription polymerase chain reaction and fluorescence in situ hybridization assays

“…However, a spectrum of histologic variants [6,[21][22][23][24] and rare diagnostically indeterminate lesions [25] have been described. In such cases, documentation of the highly specific genetic rearrangements is desirable.…”