EMPD Report

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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.

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.

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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 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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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

Prostate biopsy via the perineum is a standard method to diagnose prostate cancer. Although prostate biopsy specimens occasionally contain skin tissues, only the prostate tissue is considered. In the present case, EMPD was incidentally found in migrating skin tissues in the prostate biopsy. In this case, neither the patient nor the urologist noticed EMPD until it was pathologically confirmed.

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.

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Five- and 10-year survival in extramammary Paget's disease: A focus on wide local excision

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Five- and 10-year survival in extramammary Paget's disease: A focus on wide local excision

This study aimed to analyze extramammary Paget's disease (EMPD)-specific survival, overall survival, and recurrence rate (RR) in patients with EMPD in South Korea, with a focus on wide local excision. Based on the survival and RRs obtained, surgical treatment of EMPD with wide local excision provides fair curative resection.

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Efficacy of Abemaciclib in the Management of Refractory Metastatic Extramammary Paget’s Disease

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Efficacy of Abemaciclib in the Management of Refractory Metastatic Extramammary Paget’s Disease

Published systemic therapy options for metastatic extramammary Paget's disease have largely been anecdotal due to the rarity of this disease, which has precluded the ability to conduct clinical trials. We describe the favorable response of a 72-year-old man with extramammary Paget's disease, whose disease has been controlled with the CDK4/6 inhibitor, abemaciclib. The rationale behind the selection of this therapy is discussed.

Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease

Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease

Extramammary Paget disease (EMPD) is a frequently recurring malignant neoplasm with metastatic potential that presents in older adults on the genital, perianal, and axillary skin. Extramammary Paget disease can precede or occur along with internal malignant neoplasms. The key findings were as follows: (1) Multiple skin biopsies, including those of any nodular areas, are critical for diagnosis. (2) Malignant neoplasm screening appropriate for age and anatomical site should be performed at baseline to distinguish between primary and secondary EMPD. (3) Routine use of sentinel lymph node biopsy or lymph node dissection is not recommended. (4) For intraepidermal EMPD, surgical and nonsurgical treatments may be used depending on patient and tumor characteristics, although cure rates may be superior with surgical approaches. For invasive EMPD, surgical resection with curative intent is preferred. (5) Patients with unresectable intraepidermal EMPD or patients who are medically unable to undergo surgery may receive nonsurgical treatments, including radiotherapy, imiquimod, photodynamic therapy, carbon dioxide laser therapy, or other modalities. (6) Distant metastatic disease may be treated with chemotherapy or individualized targeted approaches. (7) Close follow-up to monitor for recurrence is recommended for at least the first 5 years.

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Prognostic value of treatment options for extramammary Paget’s disease: a SEER database analysi

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Prognostic value of treatment options for extramammary Paget’s disease: a SEER database analysi

Extramammary Paget’s disease (EMPD) is a common subtype of Paget’s disease. Still, there are lacking reports concerning its clinical features, treatment options, and prognosis. EMPD had distinct clinical features from MPD. Age, gender, chemotherapy, tumor grade and stage are independent prognostic factors for EMPD. While surgery’s protective role was supported, radiotherapy and chemotherapy could be unfavorable treatments concerning EMPD prognosis.

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Ectopic extramammary Paget disease in thoracic location

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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.

Genitourinary extramammary Paget’s disease: review and outcome in a multidisciplinary setting

Genitourinary extramammary Paget’s disease: review and outcome in a multidisciplinary setting

Extramammary Paget’s disease (EMPD) is a rare malignant disease originating from the apocrine glands involving the perineum, vulva, axilla, scrotum, and penis. To study the clinical presentation, extent of disease, efficacy of treatment, and survival outcomes of the cases in a single institution. Retrospective observation data analysis of 19 EMPD cases was performed. Demographic information, clinical management records, and histopathologic data of individual cases were obtained from the inpatient hospital data registry.

Genomic Alterations as Potential Therapeutic Targets in Extramammary Paget’s Disease of the Vulva

Genomic Alterations as Potential Therapeutic Targets in Extramammary Paget’s Disease of the Vulva

We identified all patients with primary vulvar EMPD who were treated at our institution and underwent paired tumor-normal massively parallel sequencing of 410-468 cancer-related genes (MSK-IMPACT assay). EMPD of the vulva samples sequenced from 2014 to 2019 were reviewed and somatic mutations identified, with specific focus on mutations of potential therapeutic targets. Clinical data were abstracted from electronic medical records. Microsatellite instability (MSI) was assessed by MSIscore.

Extramammary Paget Disease

Extramammary Paget Disease

Extramammary Paget disease (EMPD) is a rare dermatologic condition that frequently presents in areas where apocrine sweat glands are abundant, most commonly the vulva, although perineal, scrotal, perianal, and penile skin may also be affected. Lesions clinically present as erythematous, well-demarcated plaques that may become erosive, ulcerated, scaly, or eczematous. Extramammary Paget disease has a female predominance and usually occurs in the sixth to eighth decades of life. Professionals disagree about many aspects of EMPD, for example, the prevalence of concurrent vulvar adenocarcinoma or invasive EMPD, association with regional and distant cancers, and recurrence rates following surgical excision. Early recognition is imperative because the diagnosis is frequently delayed and there is a high incidence of associated invasive disease.

Extramammary Paget’s Disease and Melanoma: 2 Cases of Double Cancers

Extramammary Paget’s Disease and Melanoma: 2 Cases of Double Cancers

Extramammary Paget’s disease (EMPD) is a rare intraepidermal neoplastic disease. There is a well-known relationship between EMPD and underlying malignancy. However, only a few cases of EMPD and cutaneous melanoma have been reported previously. In this case report we present 2 cases of such double cancers: one as a collision tumor, the other at separate sites. We discuss the pathogenesis, treatment, and importance of a thorough clinical and radiological examination and review the literature.

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