Clinical, Pathologic and Immunohistochemical Study of Extramammary Paget's Diseases / 대한피부과학회지

ABSTRACT

BACKGROUND: Extramammary Paget's disease (EMPD) is an uncommon but distinctive tumor. Definitive diagnosis requires biopsy of the lesion and immunohistochemical staining. Immunohistochemical study using anti-cytokeratin monoclonal antibodies may be helpful for differentiating EMPD from other similar skin lesions and primary EMPD from pagetoid spread of underlying regional internal malignancy (secondary EMPD). OBJECTIVE: The purpose of this study was to identify clinical findings, histopathologic features and immunohistochemical characteristics of EMPD. METHODS: The twelve cases diagnosed as EMPD at our institution over ten years were included. Clinical records and pathology slides of the patients were reviewed retrospectively. Immunohistochemical stains for CK7, CK20, CEA, EMA, PSA, GCDFP15 and mucin stains such as PAS, d-PAS, alcian blue (pH 2.5) were done using paraffin blocks. RESULTS: 1. In most cases, EMPD developed on the scrotum of senile male patients. 2. In the pathologic findings, Paget cells were observed from the epidermis only in 2 cases, from the epidermis and intradermal skin appendages in 7 cases and from the dermis in 3 cases. 3. In all cases except for the single case associated with a transitional cell carcinoma of the urinary bladder, there were histochemical findings of alcian blue-, PAS- and immunohistochemical findings of CK7+, CK20-, CEA+, EMA+. 4. In the single case associated with transitional cell carcinoma of the urinary bladder, there were histochemical findings of alcian blue-, PAS- and immunohistochemical findings of CK7+, CK20+, CEA-, EMA-. CONCLUSION: The combination of CK7 and CK20 demonstrates these to be useful markers in distinguising primary EMPD from a pagetoid spread of extracutaneous malignancies. Namely, immunophenotypes other than CK7+/CK20- in Paget cells suggest underlying regional internal malignancy.