Shibuya Makoto, Fujisawa Kou, Yokoyama Yuichiro, Sasaki Kazuto, Ishihara Soichiro, Abe Hiroyuki, Okazaki Mutsumi
From the Department of Plastic and Reconstructive Surgery, The University of Tokyo, Tokyo, Japan.
Department of Surgical Oncology, The University of Tokyo, Tokyo, Japan.
Plast Reconstr Surg Glob Open. 2025 May 2;13(5):e6793. doi: 10.1097/GOX.0000000000006793. eCollection 2025 May.
An ileal conduit is the most commonly used urinary diversion following radical cystectomy. Malignancy of the ileal conduit rarely occurs, and Paget disease has not been reported in the peristomal skin of an ileal conduit. Herein, we describe a case of extramammary Paget disease in the peristomal skin of an ileal conduit. A 61-year-old male patient had undergone total pelvic exenteration along with ileostomy and ileal conduit diversion for colorectal cancer 30 years previously. The patient presented with a large skin ulcer around the ileal conduit in his right lower abdomen. The lesion, including the distal end of the ileal conduit, was excised based on a preoperative mapping biopsy, and the skin defect measuring 9 cm × 20 cm around the ileal conduit was simultaneously reconstructed with a pedicled anterolateral thigh flap transfer. The immunohistochemical study of the specimen revealed intraepidermal dysplastic glandular epithelial cells that were positive for CK7 and CK20 but negative for GCDFP15, suggesting that the Paget cells originated from internal malignancy. One year after the surgery, there was no recurrence of the cancer and no evidence of other malignancy. This case showed a rare presentation of secondary extramammary Paget disease in the peristomal skin of the ileal conduit. A pedicled perforator flap transfer was successfully used to immediately reconstruct a large peristomal skin defect after wide local excision.
回肠代膀胱术是根治性膀胱切除术后最常用的尿流改道术式。回肠代膀胱发生恶性肿瘤的情况罕见，且尚未见回肠代膀胱造口周围皮肤发生佩吉特病的报道。在此，我们描述一例回肠代膀胱造口周围皮肤的乳腺外佩吉特病病例。一名61岁男性患者30年前因直肠癌接受了全盆腔脏器切除术，并行回肠造口和回肠代膀胱尿流改道术。患者右下腹回肠代膀胱周围出现一个大的皮肤溃疡。基于术前定位活检切除包括回肠代膀胱远端在内的病变组织，同时采用带蒂股前外侧皮瓣转移修复回肠代膀胱周围9 cm×20 cm的皮肤缺损。标本的免疫组化研究显示表皮内发育异常的腺上皮细胞CK7和CK20阳性，但GCDFP15阴性，提示佩吉特细胞起源于内部恶性肿瘤。术后1年，癌症无复发，也无其他恶性肿瘤的证据。该病例显示了回肠代膀胱造口周围皮肤罕见的继发性乳腺外佩吉特病表现。在广泛局部切除后，成功使用带蒂穿支皮瓣转移立即修复了大面积的造口周围皮肤缺损。
