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JAAD Case Rep 2019 Feb 25;5(2):156-158. Epub 2019 Jan 25.

Advanced Dermatology Associates, LTD, Allentown, Pennsylvania.

Am J Dermatopathol 2019 Jan 29. Epub 2019 Jan 29.

Department of Dermatology, National Skin Centre, Singapore.

We describe a rare and unique case of extramammary Paget disease in the genitals with concomitant histological features of herpes virus infection. This is a very rare and interesting association that has only been reported in 1 article in the literature so far. Read More

Br J Dermatol 2019 Feb 1. Epub 2019 Feb 1.

Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.

Although carcinoembryonic antigen (CEA) and cytokeratin 19 fragment (CYFRA) are useful marker for extramammary Paget's disease (EMPD), the serum CEA and CYFRA levels are not elevated in most EMPD patients without metastasis. Therefore, further useful biomarkers are needed for the detection of EMPD including early lesions. Cell-free DNA (cfDNA) has attracted attention as an indicator of clinical conditions in several cancers, and we studied the clinical implications of cfDNA for EMPD. Read More

J Dermatol 2019 Jan 30. Epub 2019 Jan 30.

Department of Dermatology, Shinshu University School of Medicine, Matsumoto, Japan.

Gan To Kagaku Ryoho 2018 Dec;45(13):2186-2188

Division of Digestive Surgery, Dept. of Surgery, Kyoto Prefectural University of Medicine.

Anal canal adenocarcinoma with pagetoid spread has different a treatment strategy and prognosis from extramammary perianal Paget disease. We report two cases of anal canal adenocarcinoma with pagetoid spread along with a literature review. Case 1: A 69-year-old woman complained of perianal pain, redness, and erosion. Read More

Int J Gynecol Cancer 2019 Jan 23. Epub 2019 Jan 23.

Division of Gynecologic Oncology, Vul.Can MDT, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italy.

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. Read More

Skin Appendage Disord 2018 Nov 5;5(1):52-55. Epub 2018 Jun 5.

Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, Florida, USA.

Extramammary Paget disease is a rare, slow-growing intraepithelial neoplasm of the skin or its underlying appendages. It more commonly affects the apocrine glands of the axilla, vulva, perianal region, scrotum, and/or penis. It often presents as a well-demarcated, thickened, erythematous, or gray-white scaly plaque with indolent growth that may become ulcerated, crusted, papillomatous, or eczematous over time. Read More

JAAD Case Rep 2019 Jan 9;5(1):72-74. Epub 2018 Dec 9.

The University of Colorado Anschutz Medical Campus, Aurora, Colorado.

Clin Exp Dermatol 2018 Dec 11. Epub 2018 Dec 11.

Department of Dermatology, Tokyo Medical University, 6-7-1 Nishi-shinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.

J Dermatol Sci 2018 Dec 3;92(3):230-236. Epub 2018 Nov 3.

Prognosis and Statistical Investigation Committee, Japanese Skin Cancer Society, Japan; Department of Dermatology and Plastic Surgery, Kumamoto University, Japan.

Background: There are limited treatment options for advanced non-melanoma skin cancers (NMSCs). To overcome this issue, we need to conduct clinical studies, however, there is a lack of information on how many patients with advanced NMSCs are treated annually in Japan.

Objective: To investigate the actual number of advanced NMSC patients in Japan. Read More

Ir Med J 2018 06 7;111(6):772. Epub 2018 Jun 7.

Department of Urological Surgery, Tallaght Hospital, Dublin 24

We present the case of an 83-year-old man with Extramammary Paget’s disease (EMPD) of the penis. He underwent a total penectomy and histopathology confirms the association of underlying invasive high grade urothelial carcinoma. Penile EMPD is rare and can be misinterpreted for benign skin conditions. Read More

Hematol Oncol Clin North Am 2019 Feb;33(1):73-85

Department of Dermatology, The Centers for Melanoma and Cutaneous Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA 02115, USA; Dana-Farber/Brigham and Women's Cancer Center, 450 Brookline Avenue, Boston, MA 02115, USA. Electronic address:

Extramammary Paget disease (EMPD) is a rare cutaneous malignancy most commonly affecting the genitals, perineum, and perianal area of the elderly. Despite its rarity, to those impacted, the disease and its treatment can have a tremendous impact on quality of life. Commonly confined to the epidermis, EMPD can be invasive, associated with contiguous extension or upward pagetoid spread of underlying malignancy or with distant synchronous malignancy. Read More

BMC Cancer 2018 Nov 20;18(1):1142. Epub 2018 Nov 20.

Department of Gastroenterology, University of Tsukuba Hospital, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.

Background: Anal canal cancer occasionally accompanies extramammary Paget disease. Although most of them are squamous cell carcinoma, anal canal adenocarcinoma with neuroendocrine features accompanying secondary extramammary Paget disease has never been reported.

Case Presentation: Here, we report a 76-year-old man presented with pruritus in the perianal area. Read More

J Am Acad Dermatol 2018 Nov 17. Epub 2018 Nov 17.

Dept. Obstetrics & Gynaecology, Radboud university medical center, Nijmegen, the Netherlands.

Background: Vulvar Paget disease (VPD) is a rare skin disorder, considered premalignant.

Objective: To assess the clinical course, treatment schedules and the effect of invasion and treatment on recurrence and survival in patients with VPD.

Methods: Data on women with VPD were retrieved from the medical files and pathology reports in all Dutch tertiary university medical centres. Read More

Australas J Dermatol 2018 Nov 18. Epub 2018 Nov 18.

Department of Dermatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.

G Ital Dermatol Venereol 2018 Nov 12. Epub 2018 Nov 12.

Woman's Health Sciences Department, Università Politecnica delle Marche, Ancona, Italy -

Gynecol Oncol 2018 12 28;151(3):453-459. Epub 2018 Sep 28.

Department of Gynaecology, Leiden University Medical Centre, the Netherlands. Electronic address:

Background: Non-invasive vulvar Paget disease (VPD) is a rare skin disorder mainly affecting elderly women. Recently, the immune modulator imiquimod was reported as an effective treatment option. Knowledge about the immune microenvironment of VPD is lacking. Read More

BMC Cancer 2018 Sep 25;18(1):921. Epub 2018 Sep 25.

Laboratory of Cell and Molecular Tumor Biology and Bioactive Compounds, Department of Postgraduate Program in Health Science, São Francisco University (USF), Avenida São Francisco de Assis, 218, Jardim São José , Bragança Paulista, São Paulo, 12916-900, Brazil.

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. Read More

Am J Dermatopathol 2018 Sep 24. Epub 2018 Sep 24.

Department of Dermatology, Fundación Jiménez Díaz, Madrid, Spain.

Mucinous metaplasia of the vulva (MMV) is a histopathologic finding that has been reported previously in only 3 patients and needs to be distinguished from vulvar extramammary Paget disease. We report 3 additional instances of MMV associated to Zoon vulvitis and vulvar lichen sclerosus et atrophicus. Histochemical and immunohistochemical studies were performed on biopsies from erythematous and erosive vulvar lesions of 3 women aged 64, 80 and 84 years, with features of Zoon vulvitis (2 cases) and lichen sclerosus et atrophicus (1 case). Read More

CMAJ 2018 Sep;190(38):E1142

Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China

Am J Dermatopathol 2018 Oct;40(10):711-720

Consultant Histopathologist, Department of Anatomic Pathology, Hospital Universitario de Guadalajara, Guadalajara, Spain.

Background: Mammary Paget disease and extramammary Paget disease (EMPD) have different prognoses. Because they are indistinguishable on histopathological grounds, they must be distinguished on a topographical basis.

Objective: To study cases of Paget disease of the breast, which show similarities to EMPD. Read More

J Dermatol 2018 Dec 14;45(12):1456-1458. Epub 2018 Sep 14.

Department of Dermatology, Faculty of Medicine and Graduate School of Medicine Hokkaido University, Sapporo, Japan.

Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy that mainly affects the senior population. There is a relatively high risk of postoperative complications from surgery in the senior population, such as disuse syndrome, deep vein thrombosis and postoperative delirium. To prevent such postoperative complications, early ambulation is recommended. Read More

Zhonghua Fu Chan Ke Za Zhi 2018 Aug;53(8):540-546

Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.

To investigate the clinical and pathological features, diagnosis and treatment of primary vulvar Paget disease (VPD) , and analyze the related factors that may affect the recurrence. A retrospective study was carried out on 36 patients diagnosed as VPD pathologically from January 1983 to December 2017 at Peking Union Medical College Hospital, Chinese Academy of Medical Sciences. The clinical and pathological features, diagnosis, treatment and prognosis and the factors influencing recurrence rate of VPD were analyzed. Read More

Int J Dermatol 2018 09 25;57(9):e76-e78. Epub 2018 Jun 25.

Department of Dermatology, University of Arizona College of Medicine, Phoenix, AZ, USA.

Cutis 2018 Jun;101(6):422-424

Department of Pathology, University of Colorado Anschutz Medical Campus, Denver, USA.

Extramammary Paget disease (EMPD) is a malignant tumor typically found in apocrine-rich areas of the skin, particularly in the anogenital region. Some germinative apocrine-differentiating cells might exist on the trunk, preferentially in Asian individuals. Ectopic EMPD arises in nonapocrine-bearing areas, specifically the nongerminative milk line. Read More

Surg Case Rep 2018 Jul 25;4(1):81. Epub 2018 Jul 25.

Department of General Surgical Science, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma, 371-8511, Japan.

Background: Extramammary Paget's disease (EMPD) of the esophagus is a rare tumor, with most cases originating from invasive adenocarcinoma of the esophagus. Pure esophageal Paget's disease, in which no underlying invasive carcinoma component is present, is extremely rare. In this report, we describe a case of EMPD of the esophagogastric junction with no evidence of invasive carcinoma. Read More

Medicine (Baltimore) 2018 Jul;97(30):e11638

Department of Gastrointestinal Colorectal and Anal Surgery, China-Japan Union Hospital of Jilin University, Jilin, P.R. China.

Rationale: Extramammary Peget disease (EMPD) is a rare tumor, which typically occurs in the perianal regions. Perianal Paget disease (PPD) was first reported in 1893, and which has only 180 cases that have been reported in literature. The rarity of the disease means that no large studies have been made, and so the optimal treatment for this disease still remains controversial. Read More

Am J Surg Pathol 2018 Nov;42(11):1472-1479

Department of Pathology and Laboratory Medicine.

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. Read More

Eur J Dermatol 2018 Jun;28(3):351-355

Service de Dermatologie, Nantes University Hospital, Nantes, France.

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. Read More

Cancer Immunol Immunother 2018 08 25;67(8):1297-1303. Epub 2018 Jun 25.

Department of Pathology, North Hospital, University Hospital of Saint-Etienne, 42055, Cedex 2, Saint-Étienne, France.

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. Read More

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Jun;50(3):572-575

Department of Dermatology, Peking University People's Hospital, Beijing 100044, China.

Pigmented extramammary Paget's disease (PEMPD) is an uncommon intraepithelial adenocarcinoma and a rare variant of Paget's disease, characterized as a superficial pigmented scaly macule clinically and an increased number of melanocytes scattered between the Paget's cells histologically. So it may be confused clinically and histologically with melanocytic tumors, dermatitis and other dermatoses. Different therapeutic attitudes are required in this case of adenocarcinoma in situ as opposed to melanoma and dermatitis. Read More

Cancer Commun (Lond) 2018 06 19;38(1):38. Epub 2018 Jun 19.

Particle Radiation Oncology, Kyoto University Research Reactor Institute, Osaka, 590-0494, Japan.

Background: Although the most commonly recommended treatment for melanoma and extramammary Paget's disease (EMPD) of the genital region is wide surgical excision of the lesion, the procedure is highly invasive and can lead to functional and sexual problems. Alternative treatments have been used for local control when wide local excision was not feasible. Here, we describe four patients with genital malignancies who were treated with boron neutron capture therapy (BNCT). Read More

Am J Dermatopathol 2019 Jan;41(1):45-49

Department of Pathology, Wayne State University School of Medicine, Detroit Medical Center, Detroit, MI.

Extramammary Paget disease (EMPD) is a rare intraepithelial carcinoma and an uncommon variant of Paget disease affecting areas of the apocrine-rich skin of the perineum, vulva, and less commonly, axilla. Women in their sixth to eighth decades are commonly affected. It is exceedingly rare for EMPD to present on the face, chest, abdomen, or other nonapocrine sites and even more unusual for EMPD to present as a pigmented lesion. Read More

Mayo Clin Proc 2018 Jul 24;93(7):877-883. Epub 2018 May 24.

Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Mayo Clinic, Rochester, MN. Electronic address:

Objectives: To identify the rates of associated and occult cancers in patients with extramammary Paget disease (EMPD) discovered using cancer screening methods at a tertiary medical center; to propose evidence-based cancer screening guidelines at the time of diagnosis of EMPD; and to clarify terminology associating EMPD with underlying malignancies.

Patients And Methods: A retrospective review of patients with histologically confirmed EMPD presenting for care at our institution between January 1, 1992, and December 31, 2015, was performed. Both male and female patients were included. Read More

Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi 2017 Jun;31(6):714-717

Department of Burns and Plastic Surgery, West China Hospital, Sichuan University, Chengdu Sichuan, 610041,

Objective: To evaluate the effectiveness and advantages of the wide local excision for Paget's disease involing the penis and scrotum by comparing with the radical excision.

Methods: A retrospective analysis was made on the clinical data of 41 patients with Paget's disease involving penis and scrotum who met the inclusion criteria between November 2010 and August 2015. Among them, 14 patients received wide local excision (group A), and 27 patients received radical excision (group B). Read More

J Eur Acad Dermatol Venereol 2018 Nov 11;32(11):1862-1873. Epub 2018 Jun 11.

Department of Dermatology, University of Arizona and University of Arizona Cancer Center at Dignity Health, Phoenix, AZ, USA.

Extramammary Paget's Disease (EMPD) is a rare intraepithelial adenocarcinoma that classically manifests with pruritic, erythematous and scaling plaques. The clinical picture frequently mimics inflammatory or infectious conditions and is thus commonly misdiagnosed. The assessment of tumour margins is equally challenging as tumours have a propensity to spread beyond clinically visible boundaries. Read More

Cutis 2018 Apr;101(4):E19-E22

Division of Dermatology, Loyola University Chicago, Maywood, Illinois, USA.

Extramammary Paget disease (EMPD) is a rare intraepithelial adenocarcinoma. The current mainstay of treatment is wide local excision. We present the case of a 56-year-old woman with perianal EMPD that recurred 4 years after initial treatment with wide local excision with Mohs micrographic surgery tissue processing of marginal tissue. Read More

Minerva Urol Nefrol 2018 May 14. Epub 2018 May 14.

Section of Pathological Anatomy, Polytechnic University of the Marche Region, School of Medicine, United Hospitals, Ancona, Italy -

Int J Surg Pathol 2018 Oct 10;26(7):617-620. Epub 2018 May 10.

1 Indiana University, Indianapolis, IN, USA.

Introduction: Extramammary Paget disease (EMPD) of the vulva has been shown to express p16 by immunohistochemistry (IHC), however, p16 expression in the vulva and scrotum has not been extensively studied in relation to human papillomavirus (HPV) within EMPD of both the vulva and scrotum.

Design: Twenty-two cases of EMPD (vulva, 16; scrotum, 6) were found in our laboratory information system. P16 and HPV IHC were performed. Read More

Med Oncol 2018 May 9;35(6):92. Epub 2018 May 9.

Department of Dermatology, Keio University School of Medicine, 35 Shinano-machi, Shinjuku-ku, Tokyo, 160-8582, Japan.

The human epidermal growth factor receptor 2 (HER2) is recognized as an oncogene as well as a therapeutic target in various cancers. Certain patients with advanced extramammary Paget's disease (EMPD) have also been reported to express HER2, which is therefore considered a therapeutic target for EMPD. However, an accurate methodology to determine HER2-positive EMPD has not been established. Read More

Ultrasound Q 2018 Jan 5. Epub 2018 Jan 5.

Department of Radiology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea.

Extramammary Paget disease (EMPD) is a rare intraepithelial malignancy originating in areas rich in apocrine glands, such as the vulva, perineum, perianal area, scrotum, and penis. A few reports of EMPD involving the scrotum have been described in the literature. However, only few reports have described imaging features observed in patients presenting with EMPD. Read More

Curr Treat Options Oncol 2018 May 3;19(6):27. Epub 2018 May 3.

General and Visceral Surgery, Asklepios Klinik Altona, Paul-Ehrlich-Straße 1, 22673, Hamburg, Germany.

Opinion Statement: Extramammary Paget's disease (EMPD) is a rare, slow growing non-melanoma skin cancer.Diagnosis is often significantly delayed, since clinical presentation may resemble common benign dermatoses. Treatment is characterized by high recurrence rates. Read More

Acta Derm Venereol 2018 Jul;98(7):706-707

Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 700-8558 Okayama, Japan.

Clin Genitourin Cancer 2018 08 23;16(4):e899-e901. Epub 2018 Mar 23.

Department of Urology, Jiangsu Cancer Hospital, Jiangsu Institute of Cancer Research, The Affiliated Cancer Hospital of Nanjing Medical University, Nanjing, China. Electronic address:

Introduction: Metastatic extramammary Paget disease (EMPD) as a rare intraepithelial carcinoma is fatal. However, no standardized chemotherapy has been established. We provided docetaxel combined with cisplatin to EMPD patients. Read More

Photodiagnosis Photodyn Ther 2018 Sep 15;23:119-131. Epub 2018 Apr 15.

University of Florence School of Health Sciences, Department of Surgical and Translational Medicine, Section of Dermatology, P. Palagi Hospital, Viale Michelangelo 41, 50125, Florence, Italy.

Photodynamic Therapy is a procedure based on the interaction between a Photosensitizer, a light source with a specific wavelength and oxygen. The aim of this review is to provide a brief and updated analysis of scientific reports on the use of PDT with topical PS in the management of oncological, infectious, and inflammatory disorders involving mucosal and semimucosal areas, with a specific focus on diseases of dermatologic interest. Read More

Ann Chir Plast Esthet 2018 Jul 9;63(4):343-348. Epub 2018 Apr 9.

Department of plastic reconstructive and aesthetic surgery, university hospital of Picardie, 80054 Picardie cedex 1, France. Electronic address:

Objective: Perineal defects are encountered ever more frequently, in the treatment of vulvar cancers or abdominoperineal resection. The surgical treatment of vulvar cancer leads to significant skin defect. The aim of the reconstruction is not to provide volume but rather to resurface perineum. Read More

BMC Cancer 2018 04 10;18(1):403. Epub 2018 Apr 10.

Department of Urology, Shanghai 9th People's Hospital, Shanghai Jiao Tong University, School of Medicine, 639 Zhizaoju Rd, Shanghai, 200011, People's Republic of China.

Background: Extramammary Paget disease (EMPD) is a rare malignant dermatosis with poorly defined outcomes. We investigated clinical characteristics of invasive EMPD at different anatomic sites and by subject demographics to determine prognostic factors for overall survival (OS).

Methods: All patient data were collected from the Surveillance, Epidemiology, and End Results (SEER) program, 1973-2013, of the U. Read More

Hum Pathol 2018 Jul 6;77:152-158. Epub 2018 Apr 6.

Department of Pathology, Shantou University Medical College, Shantou, Guangdong, 515041; Department of Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, V5Z 1L8, Canada. Electronic address:

Paget's disease (PD) is an uncommon intraepithelial adenocarcinoma with unknown pathogenesis. There are two anatomic subtypes: mammary (MPD) and extramammary (EMPD). Little is known about their molecular characteristics. Read More

Photodiagnosis Photodyn Ther 2018 Jun 5;22:166-168. Epub 2018 Apr 5.

Institute of Photomedicine, Shanghai Skin Disease Hospital, Tongji University School of Medicine, Shanghai, China. Electronic address:

Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm arising in apocrine rich area of the skin. It is divided into primary and secondary EMPD based on whether there is an underlying malignancy, either local apocrine cancers or distant neoplasms. Therefore, all patients with EMPD should undergo an extensive and targeted cancer workup, depending on the histological staining pattern and the location. Read More