Combined Papillated Bowen Disease and Clear Cell Atypical Fibroxanthoma

Abstract

We describe a case of papillated Bowen disease (PBD), associated with a clear cell atypical fibroxanthoma (CCAFXA). The epidermal lesion showed a bowenoid papillomatous growth pattern with histologic features suggestive of infection by human papilloma virus (HPV). In the dermis a neoplasm made up by spindled or polygonal cells with wide clear cytoplasm and moderate nuclear pleomorphism was found. Immunohistochemical characteristics of these two lesions were clearly different. The atypical cells of the intraepidermal proliferation were positive for AE1-AE3 anticytokeratin antibody, EMA, p16, p53 and p63. The dermal tumor was positive for vimentin, CD10, CD68, CD99, alpha-1-antitrypsin and c-kit. Histological features and immunohistochemical profile of the dermal tumor corresponded to a CCAFXA, a very uncommon neoplasm of which only 10 cases have been reported. In situ hybridization for numerous types of HPVs was negative in both lesions.

Fig. 1

a Verrucous exophytic lesion with hyper- and parakeratosis on the surface. The growth pattern is similar to those of the anal-genital condyloma. HE, ×40. b Disorderly maturation and atypical keratinocytes associated with keratinocytes with prominent perinuclear halos reminiscent of koilocytes. HE, ×200. c, d Tumoral proliferation with solid or fascicular growth pattern placed below the dysplastic epithelium (c) and the reactive epidermis (d). In both areas the spindle or polygonal cells show wide, clear, vacuolized cytoplasm. HE, ×200. e Clear cells with moderate nuclear pleomorphism and abundant mitotic figures, some of them atypical. PAS, ×400.

Fig. 2

a Stain for p16 in most of the bowenoid lesion. The dermal tumor is negative. ×100. b Strong nuclear staining for p63 protein in the whole thickness of the dysplastic epithelium. The dermal tumor is negative. ×100. c Diffuse cytoplasmic staining for CD68 in spindle cells of the dermal neoplasm. The bowenoid lesion is negative. ×100. d Staining for CD10 at the cellular membrane in all the cells of the dermal tumor, without expression in the epidermal lesion. ×100.