CD34+ Pigmented Fibrous Proliferations: The Morphologic Overlap Between Pigmented Dermatofibromas and Bednar Tumors

Department of Pathology, Stanford University Medical Center, Stanford, CA 94305, USA.
The American Journal of dermatopathology (Impact Factor: 1.39). 11/2008; 30(5):484-7. DOI: 10.1097/DAD.0b013e3181723666
Source: PubMed


Pigmented dermatofibrosarcoma protuberans (DFSP; Bednar tumor) constitutes 5%-10% of all cases of DFSP and shows morphologic features that overlap with melanocytic and fibrous proliferations. We report 2 unusual cases of pigmented fibrous proliferations that demonstrate features of dermatofibromas and DFSP. The first case is that of a 19-year-old man with a 3-year history of a slowly growing pigmented lesion on the right arm. On clinical exam, the lesion was a 7-mm firm pigmented papulonodular lesion. The second case is that of a 31-year-old woman with a 4- to 5-year history of a slowly enlarging, asymptomatic "dark area" on the right buttock. On clinical exam, the lesion was a 2-cm darkly pigmented flat nodule. Morphologically, both lesions are primarily dermal proliferations of spindled cells admixed with pigmented dendritic melanocytes. The lesional cells trap collagen fibers at the periphery and there is basal cell hyperpigmentation. Adnexal structures are effaced, but significant trapping of subcutaneous fat is not present. By immunohistochemistry, both lesions show focal CD34 positivity but are negative for Factor XIIIa and melanocytic markers. Although overlap between standard dermatofibromas and DFSP is well documented in the literature, pigmented fibrous lesions with features of both entities are not well described.

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    ABSTRACT: The distinction between dermatofibroma (DF) and dermatofibrosarcoma protuberans (DFSP) is a well-known challenge for dermatopathologists. Immunohistochemical stains have been used to augment routine histological examination to aid in differentiating DF from DFSP. Collagen triple helix repeat containing-1 (Cthrc1) was identified as a novel gene expressed in the adventitia and neointima on arterial injury. It is indicated to be a cell type-specific inhibitor of transforming growth factor-β, which in turn impacts collagen type I and III deposition, neointimal formation, and dedifferentiation of stem cells. Cthrc1 has also been shown to be highly active and potent in degrading extracellular matrix proteins and was found to be overexpressed in several malignant tumours, such as breast cancer and malignant melanoma. To our knowledge, however, expression of Cthrc1 in DFSP and DF has not been studied before. To assess the expression of Cthrc1 in DFSP and DF and to ascertain whether Cthrc1 is superior to antibodies traditionally used in differentiating DF from DFSP. Immunohistochemical staining was performed on 23 cases of DFSP and 35 cases of DF, using antibodies to Cthrc1, CD34, factor XIIIa, CD10 and stromelysin-3 (ST3). Twenty-two of 23 (96%) DFSP samples were positive for Cthrc1, whereas 32 of 35 (91%) DF samples were negative. CD34 was expressed in most DFSPs (22 of 23, 96%), whereas it was completely negative in most cases of DF (29 of 35, 83%). Expression of factor XIIIa was found in most cases of DF (33 of 35, 94%), whereas it was completely absent in 21 of 23 (91%) DFSP cases. Expression of CD10 was found in most cases of DF (30 of 35, 86%), whereas it was completely absent in 13 of 23 (57%) DFSP cases. ST3 was expressed strongly in most cases of DF (32 of 35, 91%), whereas it was completely absent in 18 of 23 (78%) DFSP cases. The preferential Cthrc1 staining of DFSP in comparison with DF was statistically significant (P < 0·01). We confirmed that Cthrc1 is a positive marker for DFSP and that Cthrc1 staining might be more reliable than markers traditionally used. Cthrc1 was not positive in absolutely [corrected] all cases of DFSP, and combination with CD34, factor XIIIa and ST3 immunostaining could make the distinction more reliable.
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