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Cancer Immunol Immunother 2019 Apr 5. Epub 2019 Apr 5.

Pathology Department, INSERM UMR_S1165, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris (APHP), 1 Avenue Claude Vellefaux, 75010, Paris, France.

Background: Adnexal carcinomas are rare and heterogeneous skin tumors, for which no standard treatments exist for locally advanced or metastatic tumors.

Aim Of The Study: To evaluate the expression of PD-L1 and CD8 in adnexal carcinomas, and to study the association between PD-L1 expression, intra-tumoral T cell CD8 infiltrate, and metastatic evolution.

Materials And Methods: Eighty-three adnexal carcinomas were included. Read More

Intractable Rare Dis Res 2019 Feb;8(1):20-23

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

Extramammary Paget's disease (EMPD) is a rare skin malignant tumor. The prognosis of EMPD with distant metastasis is poor, however an effective therapy has not yet established. Recently, EpCAM (epithelial cell adhesion molecule, CD326) has attracted attention as both prognostic marker and therapeutic target in several cancers. Read More

Oncol Lett 2019 Mar 14;17(3):2677-2686. Epub 2019 Jan 14.

Department of Urology, Fudan University Shanghai Cancer Center, Shanghai 200032, P.R. China.

The role of human epidermal growth factor receptor 2 (HER2) amplification as a biomarker for treatment in patients with lymph node (LN)-metastatic penoscrotal extramammary Paget's disease (EMPD) was investigated in the present study. A total of 11 male patients with LN-metastatic penoscrotal EMPD were retrospectively reviewed. Positron emission tomography/computed tomography (PET/CT) was conducted prior to surgery. Read More

Indian J Dermatol Venereol Leprol 2019 Feb 26. Epub 2019 Feb 26.

Department of Dermatologic Surgery, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, China.

Extramammary Paget's disease is a rare skin malignancy, and its diagnosis requires invasive biopsy and histopathological examination. Surgery is the standard treatment for extramammary Paget's disease patients; however, as incision boundaries and the depth of tumor cell infiltration are often unclear, the postoperative recurrence rate is high. We present a case in which we used photodynamic diagnosis in combination with reflectance confocal microscopy before surgery to detect an extramammary Paget's disease lesion that was located 3 cm away from the classical lesion. Read More

Am J Dermatopathol 2018 Nov 8. Epub 2018 Nov 8.

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, TX. Dr. Yildiz is now with the Department of Pathology, Bezmialem Vakif University, Istanbul, Turkey. Dr. Ronen is now with the Department of Pathology, Medical College of Wisconsin, Milwaukee, WI. Dr. Trinidad is now with the Department of Anatomic Pathology, University of Santo Tomas Hospital Benavides Cancer Institute, Manila, Philippines.

Br J Dermatol 2019 Feb 21. Epub 2019 Feb 21.

Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.

Background: Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma affecting the genitals and axillary regions. As metastasis of these tumours is itself rare, solid disease management strategies have not been established. Serum carcinoembryonic antigen (CEA) and cytokeratin 19 fragment 21-1 (CYFRA 21-1) levels have been identified as candidate biomarkers for tumour progression in EMPD; however, neither the accuracy of, nor correlation between, these markers have been examined in EMPD patients. Read More

JAMA Dermatol 2019 Feb 20. Epub 2019 Feb 20.

Harvard Medical School, Boston, Massachusetts.

Importance: Extramammary Paget disease (EMPD), a rare intraepithelial adenocarcinoma, poses a therapeutic challenge with high postoperative recurrence rates and a limited number of effective local treatment options.

Objective: To describe the use and efficacy of a topical combination of fluorouracil and calcipotriene as a palliative therapy for refractory EMPD.

Design, Setting, And Participants: This retrospective case series of 3 women with recurrent, refractory EMPD was conducted at Beth Israel Deaconess Medical Center, Boston, Massachusetts and Washington University School of Medicine, St Louis, Missouri. Read More

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, 1-1-1 Honjo, Kumamoto, 860-8556, Japan.

Background: Although carcinoembryonic antigen (CEA) and cytokeratin 19 fragment (CYFRA) are useful markers for extramammary Paget disease (EMPD), serum CEA and CYFRA levels are not elevated in most patients with EMPD without metastasis. Cell-free (cf)DNA has attracted attention as an indicator of clinical conditions in several cancers.

Objectives: To identify further useful biomarkers for the detection of EMPD, including early lesions, and to study the clinical implications of cfDNA in EMPD. Read More

J Dermatol 2019 Apr 30;46(4):361-363. 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

Obstet Gynecol Clin North Am 2019 Mar;46(1):125-135

Department of Obstetrics and Gynecology, University of New Mexico, MSC10 5580, 1 University of New Mexico, Albuquerque, NM 87131, USA.

This article reviews the epidemiology, diagnosis, and management of vulvar preinvasive lesions, squamous cell carcinoma, and melanoma. There is an emphasis on sentinel lymph node dissection for early stage disease and advances in chemoradiation for late-stage disease. A brief review of vulvar Paget disease is also included. 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 2019 Apr 11;44(3):e61-e62. 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

BMC Cancer 2018 Nov 7;18(1):1078. Epub 2018 Nov 7.

Department of Dermatology, No.1 Hospital of China Medical University, 155N. Nanjing Street, Shenyang, 110001, People's Republic of China.

Background: Extramammary Paget's disease (EMPD), a rare skin malignancy with non-specific manifestations, is often misdiagnosed as eczema of scrotum or tinea cruris. Although the diagnosis of EMPD could be confirmed by biopsy, it can be delayed as patients are reluctant to receive invasive operations. Herein, we investigated the serum miRNA expressions of EMPD patients and compared to that of the eczema of scrotum or tinea cruris patients as well as health volunteers for potential diagnostic markers for EMPD. 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 -

Dermatol Ther 2018 11 28;31(6):e12732. Epub 2018 Oct 28.

Department of Urology, Itami City Hospital, Itami, Japan.

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 2019 Apr;41(4):e39-e42

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

J Am Acad Dermatol 2019 May 17;80(5):1215-1231.e6. Epub 2018 Sep 17.

Department of Dermatology and Pediatric Dermatology, Cutaneous Physiopathology and Integrated Center of Metabolomics Research, San Gallicano Dermatologic Institute, IRCCS, Rome, Italy.

Acquired disorders with depigmentation are commonly encountered by dermatologists and present with a wide differential diagnosis. Vitiligo, the most common disorder of acquired depigmentation, is characterized by well-defined depigmented macules and patches. Other conditions, such as chemical leukoderma, can present with similar findings, and are often easily mistaken for vitiligo. Read More

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

Dermatol Ther 2018 09 20;31(5):e12663. Epub 2018 Jul 20.

Department of Dermatology, People's Hospital of Guangxi Zhuang Autonomous Region, Nanning, China.

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 07 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 06;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 -