外陰部Paget病の膀胱転移の1例

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A very rare case of bladder metastasis of the Paget's disease of the vulva is reported. A 83-year-old woman first experienced an eczematoid eruption on the vulva 12 years earlier. It was diagnosed as Paget's disease of the vulva and treated with radiation 8 years earlier. In March, 1985 a biopsy of the vulva revealed a recurrence of Paget's disease. In August, 1986 she had a sudden attack of left flank pain. The excretory urogram showed bilateral hydronephrosis and transurethral echogram of the bladder demonstrated large nodular tumors of the bladder. Transurethral biopsies of the bladder revealed Paget's disease. Total cystourethrectomy with bilateral ureterocutaneostomy and wide skin excision of the vulva with skin transplantation were performed. The pathological specimen showed several yellowish-white nodular tumors of the bladder, which obstructed both ureteral orifices. Microscopically typical Paget's cells were seen, some of which were stained with PAS before and after amylase and with alcian blue. Postoperatively she developed a severe complication of necrosis of the small intestine due to thrombosis of superior mesenteric artery and died of panperitonitis on the 55th postoperative day. Related reports were also reviewed.