Extra mammary pagets disease

INTRODUCTION: Extra-mammary Paget's disease is a rare form of intraepithelial adenocarcinoma with a variable pattern of invasion. Vulvar Paget's disease represents 1% of all vulvar cancers. Standard treatment is surgical excision, however, the recurrence rate is high and surgery leads to anatomical, functional and sexual morbidity. Some studies suggest the efficacy of photodynamic therapy following CO2 Laser irradiation as treatment for this disease. METHODS: In the present study, ten patients with vulvar Paget's disease underwent fractional 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 lamp...

Malassezia yeast play a role in the pathogenesis of chronic dermatitis, especially in apocrine areas, by polarizing the local immunologic background to a Th2/Th17 state through aryl hydrocarbon receptor (AhR)-dependent pathways. Extra-mammary Paget's disease (EMPD) is an adenocarcinoma of apocrine origin, and except for cases associated with Malassezia yeast and their metabolites, the lesions typically develop in areas not exposed to environmental material. The purpose of this study was to investigate (a) the immunomodulatory effects of Malassezia metabolites on normal human keratinocytes (NHKCs), focusing on interleukin (IL)-17 and related cytokines/chemokines (IL-23, IL-36γ, CCL20), (b) the expression of these factors in lesion-affected skin in EMPD and (c) the activation of tumor-associated macrophages (TAMs) by these factors...

OBJECTIVES: To review the published literature on vulvar Paget's disease (VPD) molecular bases, aiming to support the need for tailored treatment in women affected by this 'orphan' tumor. METHODS: MEDLINE-PubMed and Scopus were interrogated using the following algorithm: (extramammary OR extra mammary OR vulvar) AND (paget OR pagets OR paget's) AND (molecular OR biological OR marker OR protein OR target OR expression). The inclusion criteria for papers were: peer-reviewed English-language journals, articles published in the last 30 years, studies focused on fixed research questions, quality assessment on the basis of the relevance and contribution to the selected topics...

Neutron capture therapy (NCT) is a targeted radiotherapy for cancer treatment. In this method, neutrons with a spectra/specific energy (depending on the type of agent used for NCT) are captured with an agent that has a high cross-section with these neutrons. There are some agents that have been proposed in NCT including 10 B, 157 Gd and 33 S. Among these agents, only 10 B is used in clinical trials. Application of 157 Gd is limited to in-vivo and in-vitro research. In addition, 33 S has been applied in the field of Monte Carlo simulation...

BACKGROUND: Extra-mammary Paget's disease is a rare form of intraepithelial adenocarcinoma with a variable pattern of invasion, sometimes associated with distant malignancy. Vulvar Paget's disease (VPD) represents 1% of all vulvar cancers. Standard treatment is surgical excision, however, the recurrence rate is high and surgery leads to anatomical, functional, and sexual morbidity. Some case series suggest efficacy of photodynamic (PDT) as treatment for this indication. OBJECTIVES: Our aim was to assess the efficacy and tolerance of PDT in women suffering from VPD...

BACKGROUND: Mammary and extra-mammary Paget disease is a rare form of intra-epithelial glandular neoplasm which is characteristically recurrent and necessitates multiple excisions that have an important impact on morbidity. Local immuno-modulating treatments have been applied with promising results, but the local immune markers of Paget disease have not been studied. AIM OF THE STUDY: To investigate the local immune micro-environment of Paget disease. MATERIALS AND METHODS: Sixty-four specimens from 41 patients, including cases with multiple recurrences and underlying primary neoplasm, have been studied for their expression of CD3, PD-L1 and CTLA-4...

BACKGROUND: Extra-mammary Paget's disease of the vulva (EMPDV) is an infrequent chronic disease that often recurs. The aim of the study was to assess the presence of neoangiogenesis and the expression of epithelial-mesenchymal transition (EMT) markers in EMPDV, and their potential correlation with stromal invasion. METHODS: All the women consecutively treated for EMPDV at our Institute, between January 2011 and December 2014, were studied for neoangiogenesis, analysed by microvessel density (MVD) using antibodies against CD31 and CD34...

Paget's disease is an intraepithelial adenocarcinoma arising from the apocrine gland component of the skin. Paget's disease is most common in the breast but extra mammary disease is also seen. Perianal Paget's disease is a rare form of extramammary disease with few cases reported in literature. It can be primary-arising from the skin or secondary-cutaneous metastases of anorectal or genitourinary malignancy. We hereby wish to report a case of perianal Paget's disease that presented as an eczematous lesion and was diagnosed incidentally on biopsy...

Extra mammary Paget's disease (EMPD) is a rare condition involving the vulva, anogenital region, and axilla. Vulvar disease usually presents as a slow growing well-defined itchy plaque with crustations or ulcerations over the affected area in postmenopausal women. Well-established guidelines for diagnosis and management are not available for this rare condition. Our patient is a 64-year-old postmenopausal woman with a history of similar complaints of 2 years duration, not responding to multiple topical treatments...

A 78-year-old man visited our hospital with a prolapsed hemorrhoid. He was referred to the dermatology unit due to the thickness and redness of the perianal skin. He was diagnosed as having extra mammary Paget's disease by skin biopsy. After a biopsy of the anal polyp was performed to investigate the primary site, he was diagnosed with early anal canal cancer with Pagetoid spread and underwent a radical operation. Abdominoperineal resection with skin(D2 prx D3 lymphadenectomy) was performed with perineal reconstruction using a gracilis muscle graft...

OBJECTIVE: To investigate the clinical characteristics, treatment and prognosis of primary penoscrotal extra-mammary Paget's disease (PSPD). METHODS: The clinical and pathological data of 22 cases of primary PSPD were retrospectively reviewed. Survival rate of the overall patients, the invasive patients, and the patients with positive surgical margin or negative surgical margin were analyzed with Kaplan-Meier survival curve method. RESULTS: Among all the patients with primary PSPD, the median age of onset was 64...

The patient was a 91-year-old man with change in nipple appearance, itching and redness, and a palpable breast mass. At presentation, mammary Paget's disease (PD) was clinically suspected. Skin biopsy was performed and showed epidermis invaded by Paget cells, characterized by hyperchromatic nuclei and abundant pale-staining cytoplasm. Computed tomography and mammary ultrasonography confirmed the absence of an underlying invasive carcinoma, and the patient underwent right mastectomy and sentinel lymph node biopsy (SLNB)...

No abstract text is available yet for this article.

Extramammary Paget's disease is a marginated plaque resembling Paget's disease but occurring in anogenital area, axilla or most commonly on the vulva. A 62-year-old postmenopausal woman presented with extremely pruritic plaque on the perineal skin which progressed gradually over 3 years and did not respond to any topical/systemic steroids, antibiotics, and antifungals. Examination revealed 7 × 8 cm. hypertrophic, verrucous plaque with erosions, and crusts at places. There was no evidence of visceral malignancy...

No abstract text is available yet for this article.

Apocrine carcinoma is a rare malignant adnexal neoplasm. The differential diagnosis between apocrine carcinoma and cutaneous metastasis is often difficult. Here, we report a case of locally recurrent penile apocrine carcinoma initially diagnosed as metastatic adenocarcinoma of the colon. A 75-year-old man with a history of surgical resection due to sigmoid colon cancer and penile metastasis two years prior to this study presented with a nodule at the left penile base. He underwent a wide local resection of the penile mass under a suggested preoperative diagnosis of extra-mammary Paget's disease (EMPD) associated with previous sigmoid colon cancer...

BACKGROUND: Extra-mammary Paget's disease is a rare form of superficial skin cancer. The most common site of involvement is the vulva. It is seen mainly in postmenopausal white women. Paget's disease of the vulva often spreads in an occult fashion, with margins extending beyond the apparent edges of the lesion. There is a range of interventions from surgical to non-invasive techniques or treatments. The challenges of interventions are to remove or treat disease that may not be visible, without overtreatment and with minimisation of morbidity from radical surgery...

Extra mammary Paget's disease (EMPD) is a rare condition, which affects postmenopausal women. Wide local excision may not be appropriate in elderly patients with extensive disease. It is an uncommon malignant neoplasia with a high local recurrence rate. The standard treatment is local excision of the affected area with adequate margins; however, 40~45% of cases recur after surgery within 4 years. Although surgery is currently considered the preferred primary treatment for EMPD, it has a high relapse rate due to the multifocal nature of the disease...

Extra-mammary Paget disease (EMPD) is a rare intra-epithelial carcinoma that is usually found on the apocrine-rich skin of the perineum. We report 2 cases in which EMPD was initially misdiagnosed on the initial punch biopsy as melanoma-in-situ and Bowen disease respectively. Reasons for the misdiagnoses included a rare pigmented axillary variant of EMPD in the first case and atypical bowenoid features on H&E in the second. The cases are described with a critical review of the histopathological findings, along with a review of the current literature...

Photodynamic therapy (PDT) with methyl aminolevulinate (MAL) has been suggested as a useful treatment option in a number of skin tumors, other than approved indications, and infections. However, evidence is poor because it is mainly supported by isolated case reports or small case series, often with conflicting results. To assess the effectiveness, tolerability and safety of off-label MAL-PDT in daily clinical practice in 20 Italian hospitals, a retrospective observational study of medical records of patients treated for off-label oncologic and infectious skin conditions was carried out...