Penoscrotal Paget's disease.

Paget's disease (PD) denotes an initially intra-epidermal adenocarcinoma that can later invade the dermis and metastasise. Among the extramammary forms of PD (EMPD), penoscrotal presentations are rarer than the vulvar and perianal forms. Once diagnosis has been confirmed by histopathological examination, a search for associated neoplasia must be conducted, although penoscrotal EMPD is less frequently associated with underlying neoplasia than mammary PD (MPD). The associated cancer most often involves a neighbouring organ, with prostate cancer being the most common, or in some cases consists of underlying cutaneous adnexal tumours. First-line therapy consists of surgical excision. Alternatives to surgery (imiquimod, CO2 laser vaporisation, dynamic phototherapy) may be considered in certain cases.

ALA-PDT combined with holmium laser therapy of postoperative recurrent extramammary Paget's disease.

Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm arising in apocrine rich area of the skin. Surgery is the standard treatment but relapse is common. The postoperative skin defects, penile reconstruction, functional effects and old age are also challenges for curing disease. Herein, a case of postoperative recurrent EMPD, which was treated by combination therapy of non-invasive repeatable ALA-PDT and deep penetrated holmium laser is reported. Ultrasonography monitor of lesions showed light vascularity and the formerly hypoechoic lesion disappeared after treatment.

Treatment Effects Can Mimic Recurrent Extramammary Paget Disease in Perianal Skin.

The histologic differential diagnosis of perianal Paget disease includes malignant melanoma, pagetoid spread of squamous cell carcinoma, and secondary involvement by colorectal carcinoma. While consideration of these entities is useful when establishing a diagnosis, it does not apply when patients with Paget disease undergo surveillance for recurrent disease. Treatment of perianal Paget disease consists of a combination of surgical excision with skin grafts and topical chemotherapeutic agents that induce cytologic alterations in benign cells and simulate recurrent malignancy. To evaluate the therapy-related changes and possible diagnostic pitfalls in patients with Paget disease, we reviewed 412 posttreatment tissue samples from 3 women with primary perianal Paget disease who underwent wide excision, skin grafting, and topical 5-fluorouracil therapy. Biopsy samples from engrafted skin often displayed single and clustered cells with hyperchromatic nuclei dispersed in the deep epidermis. Similar cells were scattered throughout all levels of the epidermis in biopsy samples following topical chemotherapy. The abnormal cells were negative for cytokeratin 7 (CK7) and mucicarmine in both situations. Disease ultimately recurred in all patients; some Paget cells showed classic features with eosinophilic or mucinous cytoplasm and eccentric nuclei, whereas others were smaller with less conspicuous atypia. All Paget cells showed strong, membranous CK7 staining. In short, treatment of perianal Paget disease can elicit cytologic abnormalities in benign epithelial cells that simulate the cytologic features of recurrent disease, and can diminish the atypia of Paget cells. Immunohistochemical stains for CK7 can be helpful when evaluating surveillance samples from these patients.

Photodynamic therapy following carbon dioxide laser enhances efficacy in the treatment of extramammary Paget's disease.

Surgical resection is the first-choice therapy for extramammary Paget's disease, but extensive resection is highly invasive and non-surgical treatments are sometimes preferred. Although photodynamic therapy (PDT) has been used for extramammary Paget's disease for some time, recurrence and residual tumour cells are common. In the present study, five patients with extramammary Paget's disease with a total of eight lesions first underwent carbon dioxide (CO2) laser abrasion, followed by 3 h of occlusive application of aminolaevulinic acid (ALA) and then 100 J/cm2 irradiation with a 630-nm excimer dye laser. This combination treatment regime was repeated every 2 weeks for a total of 3 times. Group 1 comprised two patients (five lesions) who received CO2 laser and ALA-PDT only. Group 2 comprised three patients (three lesions) who received CO2 laser and ALA-PDT for residual tumour cells following surgery. Follow-up examinations showed that seven lesions in five patients had not recurred after 12 months, suggesting the efficacy of the present method.