A case of invasive perianal paget’s disease mimicking pagetoid spread of internal malignancy

症例は8X歳男性.X年春より肛門部の湿疹と掻痒感を自覚していた.近医皮膚科で外用剤療法を受けていたが改善せず,同医院で生検を受け,Paget細胞を認め,X+1年Y月当科に紹介された.術前検査で隣接臓器癌は認めなかったが,術中所見で内臓癌によるPaget現象が疑われ,腹会陰式直腸切断術を施行した.術後の免疫組織学的検査はPaget現象のパターンであったが,切除標本内に明らかな内臓癌はなく最終的な病理学的診断は肛門周囲Paget病であった.術後経過良好で,術後約6年現在無再発で日常生活に支障を認めていない.

We report herein the case of invasive perianal Paget’s disease. An 8X-year-old man suffered from the eruption with itching of perianal region. He treated with ointment medicines at a dermatologic clinic. The symptom was not improved and the clinic doctor performed the biopsy of the eruption. Skin biopsy revealed Paget cells. The patient was admitted to our hospital. Imaging examinations could not detect any sign of rectal and anal cancers. Abdominoperineal resection was performed because of the long infiltration of Paget’s cell in the rectal mucosa. Histological examination of the resected specimen revealed proliferation of Paget cells, but no evidence of primary anorectal cancer was found. Although immunohistochemical findings were not typical, the final pathologic diagnosis was primary invasive perianal Paget’s disease.Both immunohistochemical findings and detection of nearby occult cancers are necessary to diagnose between perianal Paget’s disease and Pagetoid spread. Sometimes, it is difficult to differentiate two diseases in preoperative examinations.

乳房外Paget病

肛門周囲Paget癌

Paget現象

免疫組織化学検査