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.

Clear Cell Atypical Fibroxantoma: A Rare Variant of Atypical Fibroxanthoma and Review of the Literature

“…Recently, CD99, CD10 and c-kit (CD117) were described as sensitive antibodies of classic AFX. However, these antibodies have not been studied in most of the published CCAFX [2]. In our case showed positive staining with CD10.…”

“…The tumour cells were positively stained with vimentin, CD68, CD10 and CD99. Tumour cells were negatively stained with S-100 protein, pan-CK, EMA, SMA and MART-1 [1][2][3][4][5]. SmithZagone et al, [6] recently reported focal HMB-45 and MART-1 expression in the giant cells of AFX.…”

“…Clinically, it can be difficult to differentiate from other tumour without histopathological examination. Clear cell atypical fibroxanthoma (CCAFX) is rare variant of atypical fibroxantoma AFX and must be distinguished from other clear cell lessions such as metastatic renal cell carcinoma, clear cell eccrine carcinoma, sebaceous carcinoma and clear cell sarcoma (malignant melanoma of soft part) and clear cell hidradenocarcinoma [1][2][3].…”

“…The neoplastic cells were stained positively with CD68 and CD10 and were stained negative with S-100, Melan A, muscle-specific actin, or pan-cytokeratin. CCAFX should be differantiated from dermal neoplasms, either primary or metastatic, composed of clear cells such as squamous cell carcinoma, basal cell carcinoma, balloon cell malignant melanoma, tricholemmal carcinoma, adnexal carcinoma, renal cell carcinoma, clear cell sarcoma, cutaneous leiomyosarcoma, dermal pleomorphic liposarcoma, chordoma with cutaneous metastase or direct cutaneous extension, parachordoma and clear cell dermatofibroma [2][3][4]. The lack of S-100, Melan A and HMB-45 staining in CCAFX excludes clear cell (balloon cell) malignant melanoma, which is also a superficial, dermal tumour.…”

Lobulated Bowen's Disease with a Clear Cell Change

“…The clinical feature of our case differs from that of papillated Bowen's disease in that the surface was not verrucous or hyperkeratotic, moreover, there was no papillomatosis on a histopathological examination12. Unusual cases of melanocytic nevi having a similar clinical appearance have been reported as “lobulated melanocytic nevi”56.…”