This study aimed to compare surgical and oncologic outcomes of margin-controlled surgery vs traditional surgical excision for genital extramammary Paget's disease (EMPD) in females.
Ectopic extramammary Paget disease in thoracic location
Extramammary Paget disease (EMPD) is a rare skin cancer that affects areas with a high concentration of apocrine glands including genital, axillary, and anal skin. When it affects other locations it is called ectopic extramammary Paget disease (E-EMPD) and is uncommon. To date, there are only 45 case reports to the best of our knowledge. The clinical manifestation is typically a soft, red or bright pink patch or plaque with scattered white islands of hyperkeratosis and erosion. Diagnostic confirmation requires conventional histology with immunohistochemistry. The importance of immunohistochemical staining for the diagnosis of primary neoplasia, without underlying malignancy, is highlighted. We report the first Latin American confirmed case, to our knowledge, of primary E-EMPD in a 55-year-old man with a 1-year history of asymptomatic thoracic plaque.
Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy
Background: Perianal Paget’s disease (PPD) is a rare intraepithelial adenocarcinoma of the anal margin. Primary PPD likely represents intra-epithelial neoplasm from an apocrine source, whereas secondary disease may represent“pagetoid” spread from an anorectal malignancy.
Case presentation: Histologic CDX-2 and CK20 are hallmark markers for colorectal-derived Paget’s cells. Interestingly, our primary PPD patient presented both positive and no internal malignancy was identified. In addition, a negative CK7 marker was observed in our case in contrast with previously reported. Surgical excision is the standard treatment; however, previous studies have demonstrated good response with Imiquimod 5% cream in patients with vulval extramammary Paget disease (EMPD). The efficiency of Imiquimod treatment for PPD has not been well described. Our PPD patient was successfully treated using Imiquimod 5% cream.
Conclusions: This study describes a primary cutaneous PPD patient CDX-2+/CK20+/CK7- without invasion of the dermis and no associated colorectal carcinoma effectively treated using topical Imiquimod therapy, suggesting that Imiquimod might potentially be considered as a first-line treatment for PPD.
Intraoperative Immunostaining for Cytokeratin-7 During Mohs Micrographic Surgery Demonstrates Low Local Recurrence Rates in Extramammary Paget's Disease
Extramammary Paget’s disease (EMPD) is a rare intraepithelial malignancy typically occurring in the groin and axillary regions. Given its indolent growth pattern and clinical resemblance to inflammatory skin conditions, diagnosis of EMPD is often delayed. Reported recurrence rates for standard surgical treatments including wide local excision, vulvectomy, and abdominoperineal resection are high, ranging from 22% to 60% in the largest retrospective cohort of patients with EMPD treated with Mohs micrographic surgery (MMS) demonstrating local recurrence rates of 26% overall, 16% for primary disease, 50% for recurrent disease, and a salvage rate for recurrent disease after MMS (overall cure rate) of 100%
The authors hypothesize that the increased recurrence rates for EMPD after MMS, as compared to other types of skin cancer, are due to difficulties in recognizing tumor cells on routine hematoxylin and eosin staining. Indeed, the authors have previously shown a case of EMPD in the axilla with irregular yet contiguous finger-like microscopic extensions that were not seen on H&E but identified on sections stained for cytokeratin-7(CK-7). The use of intra-operative immunohistochemistry for CK-7 during MMS for EMPD is described in case studies and small single-center cross-sectional studies.
Multicentric Primary Extramammary Paget Disease: A Toker Cell Disorder?
Toker cells are epithelial clear cells found in the areolar and nipple areas of the breast, vulvar region, and other apocrine gland–bearing areas of the skin. Toker cells have been implicated in the pathogenesis of clear cell papulosis, cutaneous hamartoma with pagetoid cells, and rare cases of primary extramammary Paget disease (EMPD) but not in secondary EMPD with underlying adenocarcinoma. The pathogenesis of primary EMPD is not well defined. We report a case of multicentric primary EMPD with evidence of Toker cell proliferation and nonaggressive biologic behavior in a 63-year-old white man. A detailed description of the morphologic and biologic features of Toker cells and their possible carcinogenetic links also are discussed. Based on the observation and follow-up of our patient, we hypothesize that multicentric primary EMPD starts with Toker cell hyperplasia and can potentially evolve to carcinoma in the genital region.
Extramammary Paget disease (EMPD), which was first described by Crocker1 in a patient with erythematous patches on the penis and scrotum, is morphologically identical to mammary Paget disease (MPD) of the nipple. The principal difference between EMPD and MPD is anatomic location.