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Topical Imiquimod in Primary Cutaneous Extramammary Paget’s Disease: A Systematic Review

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Topical Imiquimod in Primary Cutaneous Extramammary Paget’s Disease: A Systematic Review

The main treatment for extramammary Paget’s disease (EMPD) is still surgery, but this neoplasm usually spreads beyond the clinical surroundings of the lesion, and achieving histopathologically free margins can be challenging and requires mutilating surgery. Moreover, the recurrence rate of the disease is high, reflecting its multifocal nature. Topical immunotherapy could be an alternative treatment for EMPD. The aim of this systematic review was to assess the effectiveness of topical imiquimod in the clinical response of EMPD patients and to describe the management of topical imiquimod in EMPD. Learning about non-surgical treatments such as topical imiquimod can help clinicians manage EMPD and find a balance between disease resolution and treatment morbidity.

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TRPS1 expression in primary and secondary extramammary Paget diseases: An immunohistochemical analysis of 93 cases

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TRPS1 expression in primary and secondary extramammary Paget diseases: An immunohistochemical analysis of 93 cases

Extramammary Paget disease (EMPD) predominantly manifests de novo as primary EMPD, with less than 30 % of cases associated with underlying internal malignancy (secondary EMPD). Differentiating primary from secondary EMPDs based solely on histopathology poses challenges, often necessitating supplementary screening, such as endoscopy or imaging studies, to definitively exclude underlying carcinomas like colonic adenocarcinoma. Recently, TRPS1 immunohistochemistry, initially identified as a sensitive and specific marker for carcinomas and mesenchymal tumors of mammary origin, has been proposed for EMPD. In this study, we conducted a systematic assessment of TRPS1 expression across 93 EMPD cases, comprising 82 primary EMPDs and 11 secondary EMPDs. Upon excluding cases of perianal primary EMPDs, the sensitivity and specificity of TRPS1 for primary EMPDs reached 100 %. Our findings suggest that TRPS1 expression holds notable sensitivity and specificity for primary EMPDs, particularly when arising from non-perianal cutaneous sites. Hence, in suitable clinical contexts, TRPS1 immunohistochemistry may emerge as a promising and valuable tool for distinguishing primary and secondary EMPDs.

Extramammary Paget's Disease of the Glans Penis Secondary to a Recurrent Urothelial Carcinoma of the Bladder

Extramammary Paget's Disease of the Glans Penis Secondary to a Recurrent Urothelial Carcinoma of the Bladder

A 71-year-old man presented with an erythematous plaque around the urinary meatus that had been slowly enlarging for the past eight months. Six months before presentation, the patient was treated based on an impression of eczema. He had a history of radical cystectomy with ileal conduit urinary diversion for urothelial carcinoma of the bladder three years previously. The resected margin was negative and no recurrence had been detected afterward. Physical examination revealed a scaly, erythematous plaque around the urethral orifice.

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Network meta-analysis of treatments for perineal extramammary paget’s disease: Focusing on performance of recurrence prevention

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Network meta-analysis of treatments for perineal extramammary paget’s disease: Focusing on performance of recurrence prevention

Perineal extramammary paget’s disease (EMPD) is characterized with high recurrence rate. Although numerous therapeutic measures for this disease have been reported so far, it is unknown whether there is significant difference in their recurrence-preventing efficiency. This study aims to compare the recurrence outcomes of reported perineal EMPD treatments. We searched public databases of for published studies concerning perineal EMPD treatments. After screening by inclusion and exclusion criteria, we extracted the data relevant to recurrence rate, and conducted network meta-analysis (NMA) by using Bayesian random-effects approach. Our analysis included 29 previous studies (involving both male and female patients) and 11 treatment designs which are wide local excision (WLE), local excision (LE), Mohs micrographic surgery (MMS), radiotherapy (RT), radical vulvectomy (RV), photodynamic therapy (PDT), lasers (LS), imiquimod, and WLE+RT, WLE+PDT, WLE+LS. MMS has the best performance on reducing perineal EMPD recurrence.

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Radiotherapy in Extramammary Paget’s disease

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Radiotherapy in Extramammary Paget’s disease

Extramammary Paget’s disease (EMPD) is a rare and heterogeneous clinical picture usually recorded on apocrine gland-bearing epidermis areas in extramammary sites, showing a potential invasive evolution and poor prognosis. At diagnosis, it could appear as a primary tumor with secondary co-exhisting visceral malignancies like in anus, male or female genital system, which should be assessed. This rare epidermal carcinoma generally occurs in individuals older than 60 years mainly in Caucasian female and Asian male ethnicity. Although the pathophisiology has been well established, its management is still controversial and requires a multidisciplinary team approach. There are several therapeutic strategies. Among them, radiotherapy is accounted as an effective option in primary or in adjuvant timing but up to now no definitive role has been assessed. Data by case series, case reports and retrospective analyses have provided results on the positive benefit of HDR brachytherapy or external beam radiotherapy (EBRT) mainly in very old patients not suitable for extended surgery, which remains the main treatment care. Long course radiotherapy is a big concern in elderly patients due to logistics and care assistance deficiencies, which makes their access difficult to the radiation centres. Thus, hypofractionated radiotherapy regimens could be a good compromise problem solving to provide excellent local control within a short treatment time. Herein, we present a case of EMPD occurring in a 80 years old woman with vulvo-perineal extension successfully treated with hypofractionated external beam radiotherapy as primary treatment using a schedule 300cGy/fr/51 Gy in 17 treatment days.

Prognostic Factors of Extramammary Paget’s Disease: A Retrospective Study at a Medical Center in Taiwan

Prognostic Factors of Extramammary Paget’s Disease: A Retrospective Study at a Medical Center in Taiwan

Extramammary Paget's disease (EMPD) is a rare, slow growing intra-epidermal malignant neoplasm that arises in areas rich in apocrine glands. Several common sites of occurrence have been reported, including the vulva, perianal region, perineum, and scrotum. Most relevant studies rely on small data bases. Our objective was to evaluate prognostic factors of EMPD patients at a single medical center in Taiwan.

Identification of Predictive Factors for Post-operative Recurrence and Clinical Outcomes of Primary Vulvar Extramammary Paget Disease

Identification of Predictive Factors for Post-operative Recurrence and Clinical Outcomes of Primary Vulvar Extramammary Paget Disease

This study aimed to identify clinicopathological factors predicting recurrence and prognosis in patients with vulvar extramammary Paget disease (VPD). Forty-five patients were analyzed, revealing that 40% had resection margin (RM) involvement, 26.7% received adjuvant radiation therapy (RT), and 22.2% experienced recurrence. Recurrence rates were higher in patients who underwent less extensive surgeries like wide local excision or simple vulvectomy compared to those who had radical vulvectomy. Positive RM involvement was found to be an independent factor for poorer recurrence-free survival (RFS), while adjuvant RT significantly improved overall survival. In conclusion, positive RM involvement predicts worse RFS, the type of surgery influences recurrence rates, and adjuvant RT can enhance prognosis in VPD patients.

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Treatment of Extramammary Paget Disease and the Role of Reflectance Confocal Microscopy: A Prospective Study

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Treatment of Extramammary Paget Disease and the Role of Reflectance Confocal Microscopy: A Prospective Study

Extramammary Paget disease (EMPD) poses treatment challenges. Invasive and non-invasive treatment modalities exist with variable success reported. Reflectance confocal microscopy (RCM) is emerging as an adjuvant diagnostic tool.

EMPD is challenging and lesion demarcation is of the utmost importance. Using a staged surgical excision approach, the mean margins needed were 1.8 cm, less than previously reported. Non-surgical modalities including RT, imiquimod or PDT can be considered if surgery is not pursued. RCM is a valuable non-invasive imaging modality for the management of EMPD.

Hypopigmentation as a diagnostic clue in primary extramammary Paget disease: Case report and short literature review

Hypopigmentation as a diagnostic clue in primary extramammary Paget disease: Case report and short literature review

Primary extramammary Paget disease (EMPD) is a rare malignant tumour occurring in apocrine gland-bearing areas, such as the genitals (mean age at diagnosis 60–70 years).1 Clinically, EMPD exhibits well-circumscribed, erythematous and scaly patches or plaques often mimicking inflammatory skin disorders, but occasional pigmentary changes such as hypo- or hyperpigmentation are reported.

Extramammary Paget's disease: A case report

Extramammary Paget's disease: A case report

A 72-year-old man was admitted to the hospital with a genito-femoral lesion of the skin. The lesion started in the right groin and increased slowly in size for 10 years. It was oozing intermittently. ln the last 1 1/2 years he had observed a papillary growth on the right thigh adjacent to the inguinal fold. There had been considerable pain but no itching. The lesion had been treated for years as "eczema" or mycotic infection with a great number of topical agents.

Extramammary Paget Disease: a Therapeutic Challenge, for a Rare Entity

Extramammary Paget Disease: a Therapeutic Challenge, for a Rare Entity

Extramammary Paget disease (EMPD) is a rare entity which is more frequently localized at the vulva, though it only accounts for 1–2% of vulvar neoplasms. It is a primary cutaneous adenocarcinoma whose cell of origin is still a matter of controversy: it can either arise from apocrine/eccrine glands or from stem cells. The diagnosis demands a biopsy and entails a histopathological analysis by which cells show similar characteristics as breast Paget disease.

Treatment approach can entail surgery, radiotherapy, photodynamic therapy, systemic chemotherapy, and topical chemotherapy. For metastatic disease, many different chemotherapy regimens have been explored and even targeted therapy can play an important role in this disease. Since almost 30–40% of patients overexpress HER-2, trastuzumab and anti-HER-2 therapies can be employed in this setting.

Due to its low incidence, there is almost no specific evidence on therapeutic interventions for this disease. Thus, there is a neat unmet need for molecular characterization of EMPD and diagnostic tools that allow clinicians to guide treatment both in the early and in the advanced disease settings. In this review, we aim to summarize available evidence about diagnosis and treatment of EMPD, both localized and metastatic, and to provide a comprehensive analysis that may help clinicians for therapeutic decisions.

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Role of androgen signaling in androgen receptor-positive extramammary Paget's disease: establishment of organoids and their biological analysis as a novel therapeutic target

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Role of androgen signaling in androgen receptor-positive extramammary Paget's disease: establishment of organoids and their biological analysis as a novel therapeutic target

Extramammary Paget’s disease (EMPD) is a rare intraepithelial adenocarcinoma that mainly affects the anogenital and axillary regions. Although its etiology has not been fully elucidated, there is evidence that androgen receptors (AR) are expressed in most cases of EMPD. However, the role of androgen signaling in the pathogenesis of EMPD remains unclear. Our results indicate that androgen signaling is a key pathway involved in the growth of AR-positive EMPD. Therefore, androgen signaling inhibition may be a novel treatment option for EMPD patients who require systemic therapy.

Clinicopathological significance of androgen receptor expression in extramammary Paget disease: An analysis of 92 patients

Clinicopathological significance of androgen receptor expression in extramammary Paget disease: An analysis of 92 patients

Extramammary Paget disease (EMPD) is a rare cutaneous malignant neoplasm arising in apocrine gland-rich areas. Although – like normal apocrine glands – EMPD frequently expresses androgen receptor (AR), the clinical significance of AR expression remains unclear. The present study investigated the clinicopathological impact of AR expression in EMPD. Immunohistochemistry for AR was performed in a retrospective cohort of 92 EMPD patients with 108 EMPD lesions, including 102 primary lesions, five lymph node [LN] metastases and one local recurrence. These results clearly indicate that increased AR expression is associated with malignant progression of EMPD and that androgen blockade might be an effective therapy. Furthermore, AR expression assessed by immunohistochemistry may have potential for prediction of LN metastasis and local recurrence in EMPD.

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Mohs surgery for female genital Paget's disease: a prospective observational trial

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Mohs surgery for female genital Paget's disease: a prospective observational trial

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.

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A clinicopathological analysis of forkhead box A1 (FOXA1) and estrogen receptor alpha expression in extramammary Paget’s disease

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A clinicopathological analysis of forkhead box A1 (FOXA1) and estrogen receptor alpha expression in extramammary Paget’s disease

Extramammary Paget’s disease (EMPD) is a neoplastic skin disease of unknown etiology. EMPD is frequently associated with forkhead box A1 (FOXA1) expression, which correlates with the expression of estrogen receptor alpha (ER). FOXA1 regulates the transcriptional activity of ER and may function cooperatively in the tumorigenesis of breast cancer. In the present study, we confirmed that EMPD was frequently associated with FOXA1 expression. However, ER expression varied between patients and did not always coincide with FOXA1 expression. No clear relationship was observed between ER expression, the intensity of ER staining, or EMPD metastasis and prognosis. However, the results indicate that hormone-dependent cancer therapy may be effective in patients with ER-positive EMPD.

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Incidental finding of extramammary Paget's disease during active surveillance for early-stage prostate cancer in a prostate biopsy

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Incidental finding of extramammary Paget's disease during active surveillance for early-stage prostate cancer in a prostate biopsy

Skin tissue contamination within transcutaneous visceral organ biopsies is seldom found. We encountered a rare case of extramammary Paget's disease incidentally diagnosed by prostate biopsy during active surveillance for prostate cancer. Although the perianal region is a common site of extramammary Paget's disease, early-stage extramammary Paget's disease is often asymptomatic. Thus, during a transcutaneous biopsy, it is important to consider the appearance of the skin and the pathological features of migrating skin tissue.

Extramammary Paget Disease: a Therapeutic Challenge, for a Rare Entity

Extramammary Paget Disease: a Therapeutic Challenge, for a Rare Entity

Extramammary Paget disease (EMPD) is a rare entity which is more frequently localized at the vulva, though it only accounts for 1–2% of vulvar neoplasms. It is a primary cutaneous adenocarcinoma whose cell of origin is still a matter of controversy: it can either arise from apocrine/eccrine glands or from stem cells. The diagnosis demands a biopsy and entails a histopathological analysis by which cells show similar characteristics as breast Paget disease.

Doença de Paget vulvar: uma série de casos no sul do Brasil

Doença de Paget vulvar: uma série de casos no sul do Brasil

A doença de Paget vulvar é rara e manifesta-se clinicamente como lesão cutânea eritematosa pruriginosa com áreas de hiperqueratose. O presente relatório descreve os dados de diagnóstico, manejo e resultados de uma série de casos de mulheres diagnosticadas com doença de Paget vulvar em um hospital terciário no sul do Brasil. A doença de Paget vulvar tem uma morbidade significativa e os dados disponíveis são limitados, especialmente no Brasil. Devido à raridade da doença, não há ensaios clínicos randomizados disponíveis na literatura e, portanto, é difícil comparar os resultados do tratamento cirúrgico com outras modalidades terapêuticas.

Extramammary Paget’s Disease Manifested by Intraepithelial Adenocarcinoma of the Vulva and Anus Combined with Invasive Adenocarcinoma of the Ampullary Part of the Rectum

Extramammary Paget’s Disease Manifested by Intraepithelial Adenocarcinoma of the Vulva and Anus Combined with Invasive Adenocarcinoma of the Ampullary Part of the Rectum

The author of the given paper describes an unusual combination of two diseases: extramammary Paget’s disease manifested by intraepithelial adenocarcinoma of the vulva and anus combined with invasive adenocarcinoma of the ampullary part of the rectum and describes the atypical manifestations of these diseases.

The role of sentinel lymph node biopsy in extramammary paget disease

The role of sentinel lymph node biopsy in extramammary paget disease

Sentinel lymph node biopsy is increasingly used to detect subclinical nodal metastases in extramammary Paget disease. We performed a comprehensive systematic review of the literature to further explore the role of sentinel lymph node biopsy in extramammary Paget disease. Sentinel lymph node biopsy is a useful tool to screen for subclinical nodal metastases in invasive disease, and can be used to help guide clinical management.

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