A CASE OF PERIANAL PAGET'S DISEASE WHICH SPREAD TO PERINEAL LESION

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  • 外陰部に拡がった肛門周囲Ｐａｇｅｔ病の１例

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Abstract

An 82-year-old woman came to our hospital with the chief complaint of vulvar erythema and itching. Although Pagetoid cells with positive immunostaining for cytokeratin (CK) 20 were observed, no rectal or anal tumors were detected. However, under the diagnosis of Pagetoid spread associated with rectal or anal cancer, total pelvic excision was performed. The final histopathological diagnosis was primary extramammary Paget's disease with positive staining for CK20 and negative staining for gross cystic disease fluid protein 15 (GCDFP15). Generally, perianal Paget's cells with finding of CK20+/GCDFP15- suggest the presence of advanced ano-rectal carcinoma, and primary Paget's disease with CK20+/GCDFP15- is very rare. When perianal Paget's disease with the CK20+/GCDFP15- phenotype and without clear preoperative evidence of rectal or anal cancer, the operation should be selected taking into consideration the rate of progression of the disease, the general condition of the patient, and the radicality of the operation.

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