Giant cell fibroblastoma

Harvell JD, Kilpatrick SE, White WL. Histologic relations between giant cell fibroblastoma and dermatofibrosatcoma prom-berans CD34 staining showing the spectrum and a simulator. Am J Dermatopathol. 1998 20 339-345. 

Goldblum JR. Giant cell fibroblastoma a report of three cases with histologic and immunohistochemical evidence of a relationship to dermatofibrosarcoma protuberans. Arch Pathol Lab Med. 1996 120 1052-1055. 

Terrier-Lacombe MJ, Guillou L, Maire G, et al. Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children a clinicopathologic comparative analysis of 28 cases with molecular data—a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol. 2003 27 27-39. 

Diwan AH, Skelton III HG, Horenstein MG, et al. Dermatofibrosarcoma protuberans and giant cell fibroblastoma exhibit CD99 positivity. J Cutan Pathol. 2008 35 647-650. 

Pre-B-ALL, AML (M7), alveolar soft part sarcoma, congenital and infantile fibrosarcoma, inflammatory fibrous polyp of GIT, breast fibroadenoma, giant cell fibroblastoma, juxtaglomerular cell tumor, superficial acral fibromyxoma... 

Molecular Detection of Dermatofibrosarcoma Protuberans and Giant Cell Fibroblastoma... 

Dermatofibrosarcoma protuberans and its pediatric counterpart giant cell fibroblastoma are two tumors sharing many histological, immunohistochemical and cytogenetic features. 

Giant cell fibroblastoma is a rare pediatric dermal or subcutaneous slowly growing tumor with a predilection for children under 10 years of age. Histologically the tumor consists of proliferating spindled cells mixed with multinudeated giant cells surrounded by solid fibromyxoid stroma dissected by vascular like spaces. 

Dermatofibrosarcoma protuberans and giant cell fibroblastoma share the same immunohistochemical profile and show positive reaction with the specific antibodies to CD34, PDGF and vimentin. Both tumors reveal low or intermediate malignant behavior and tens to local recurrence after incomplete excision and characterized by very low metastatic tendency, which generally occurs after an inadequate surgical treatment. 

Dermatofibrosarcoma protuberans and giant cell fibroblastoma are associated with the novel and specific unbalanced t(17 22)(q22 ql3) translocation generating the COLIAI-PDGFB fusion transcript, occasionally with a supernumerary ring chromosome r (17 22) as a result of this translocation. 

As the t(17 22)(q22 ql3) translocation is specific for both tumors and has not been described in association with any other benign or malignant lesions, the amplification of the COLlAl-PDGFB-fusion transcript can be used as a molecular marker to distinguish dermatofibrosarcoma protuberans and giant cell fibroblastoma from other tumors with similar morphology. 

Kashima, A., yamashita. A., Moriguchi, S. et al. (2006) Detection of COLIAI-PDGFB fusion transcripts and platelet-derivated factor a and P receptors in giant cell fibroblastoma of the postsacrococcygeal region. Br J Dermatol, 154, 983-987. 

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