PMC3804824_01
Gross appearances of (a) peristomal skin lesion and (b) en-bloc extirpated specimen including the peristomal skin and right ureter (b). The peristomal skin included erythematous, erosive, eczematous and circinate lesions, which resembled Paget's disease, but the surgical margin was free from these lesions. The ureter cut open showed extensive roughness on the mucosal surface. *means exit of the right ureter,   means navel,  means planned resection line.
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Under a diagnosis of a peristomal Paget phenomenon of UC, we performed en-bloc resection of the remaining ureter and peristomal skin with a sufficient normal margin (Figure 1) and percutaneous nephrostomy, followed by reconstruction of the abdominal wall to wrap the exposed fascia with surrounding excess skin.
Light microscopic appearance of pagetoid lesions. (a) Junction of the right ureter and peristomal skin. Most of the junction was eroded. In the residual intact junction, pagetoid cells spread into the peristomal epidermis directly from the ureteral mucosa. (hematoxylin-eosin stain, 4x and 100x). (b) Intraepidermal pagetoid lesion in the peristomal skin. (hematoxylin-eosin stain, 40x and 200x). (c) Immunohistochemistry with anti-HER2, anti-CK7 or anti-CK20 antibody. The presence of both CK7 and CK20 is characteristic of secondary Paget's disease. Expression of HER2 implies an epidermotropic ability of pagetoid tumor cells (hematoxylin-eosin or immunoperoxidase stain, 40x).
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Pagetoid cells had also spread into the peristomal epidermis directly from the ureteral mucosa (Figure 2a) and were present among p63-positive cells (i.e. epidermal basal cells), which showed that pagetoid cells had spread to the basal layer of the epidermis. The surrounding stroma showed prominent congestion and infiltration of lymphocytes and histiocytes, but neither via-vessel extension nor direct invasion of neoplastic cells (Figure 2b). In immunohistochemistry, the cells in the epidermis and ureteral mucosa were positive for CK7 and CK20 (Figure 2c) and pagetoid cells were positive for HER2 (Figure 2c).