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Cell Res 2020 May 26. Epub 2020 May 26.

State Key Laboratories for Agrobiotechnology and Beijing Advanced Innovation Center for Food Nutrition and Human Health, College of Biological Sciences, China Agricultural University, Beijing, 100193, China.

Mammary and extramammary Paget's Diseases (PD) are a malignant skin cancer characterized by the appearance of Paget cells. Although easily diagnosed, its pathogenesis remains unknown. Here, single-cell RNA-sequencing identified distinct cellular states, novel biomarkers, and signaling pathways - including mTOR, associated with extramammary PD. Read More

Dermatology 2020 Apr 14:1-7. Epub 2020 Apr 14.

Division of Dermatology, Rabin Medical Center-Beilinson Hospital, Petah Tikva, Israel.

Introduction: Surgery is commonly regarded as the mainstay of treatment of extramammary Paget disease (EMPD); however, nonsurgical approaches have gained popularity in recent years.

Objectives: To review the published evidence for the efficacy and safety of nonsurgical modes of therapy for EMPD.

Methods: A systematic review and meta-analysis of nonsurgical EMPD treatments was performed. Read More

Actas Dermosifiliogr 2020 May 9;111(4):306-312. Epub 2020 Apr 9.

Servicio de Cirugía Plástica, Hospital Universitari de Bellvitge, Barcelona, España.

Background And Objective: Extramammary Paget disease (EMPD) has seldom been studied in Mediterranean populations. We aimed to review the characteristics of our patients with EMPD, the presence of a neoplasm in continuity, and the long-term course of the disease.

Patients And Methods: Retrospective observational study of 27 patients diagnosed with EMPD between 1990 and 2015. Read More

Cancers (Basel) 2020 Mar 29;12(4). Epub 2020 Mar 29.

Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.

Extramammary Paget's disease (EMPD) is a neoplastic skin disease of indeterminate origin with an unknown genetic cause. We performed a comprehensive genetic analysis or targeted gene sequencing in 48 patients with EMPD. We identified mutations, a fusion gene, and somatic hotspot mutations in the promoter region in 11 of the 48 EMPD patients (11/48, 23%). Read More

Am J Dermatopathol 2020 Mar 6. Epub 2020 Mar 6.

Departments of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, Chapel Hill, NC.

The PD-1/PD-L1 pathway plays a critical role in the physiologic inhibition and modulation of the immune response in normal tissue. Many tumors evade immune detection and response by upregulating PD-L1 expression. Humanized monoclonal PD-1 and PD-L1 antibodies have proven as both tolerable and effective treatment in many neoplasms. Read More

Plast Reconstr Surg Glob Open 2020 Jan 20;8(1):e2602. Epub 2020 Jan 20.

Department of Plastic and Reconstructive Surgery, Japanese Red Cross Society Wakayama Medical Center, Wakayama-Shi, Wakayama, Japan.

An 83-year-old Japanese man who had been aware of a tumor near his anus for 2 years underwent tumor resection. Although he was diagnosed with basal cell carcinoma (BCC), extramammary Paget disease (EMPD) was also accidentally found in the same specimen. In the pathological histology of EMPD, there were large round cells with ample cytoplasm spread in the epidermis; these cells were positive for cytokeratin 7 and gross cystic disease fluid protein 15. Read More

Iran J Pathol 2020 ;15(1):48-52

School of Medicine, Metabolic Diseases Research Center, Qazvin University of Medical Sciences, Qazvin, Iran.

Extramammary Paget's disease and vulvar intraepithelial neoplasia are common lesions of vulve, but synchronous occurrence is rare in a same location. Herein we describe a concurrence of primary cutaneous extra mammary Paget's disease and squamous cell carcinoma in situ of vulva in an Iranian women. A 59 year old woman, initially presented to Kosar teaching hospital gynecology clinic April 2017 with a single, well defined, scaly, white ulcerated mass, 2 cm in diameter on right minor labia, but other examinations were normal. Read More

Arch Plast Surg 2020 Jan 15;47(1):83-87. Epub 2020 Jan 15.

Department of Surgery, Yonsei University College of Medicine, Seoul, Korea.

Extramammary Paget disease (EMPD) is an uncommon malignancy that occurs in apocrine gland-rich areas of the body. EMPD of the axilla is rare, but a few cases have been reported. Some cases of EMPD have been reported with underlying apocrine adenocarcinoma; rarely, mammary-type ductal carcinoma can accompany EMPD. Read More

Oncotarget 2019 Nov 19;10(62):6647-6650. Epub 2019 Nov 19.

Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.

Metastatic extramammary Paget's disease is a rare adenocarcinoma with poor prognosis. Several reports of human epidermal growth factor receptor 2 alterations point to its pathogenic role in the disease. However, the occurrence of treatment resistance to anti-HER2 therapy demand the need for further knowledge. Read More

JMIR Res Protoc 2019 Dec 3;8(12):e15026. Epub 2019 Dec 3.

U1189 - Image Assisted Laser Therapies for Oncology, Inserm, Centre Hospitalier et Universitaire de Lille, Université de Lille, Lille, France.

Background: Extramammary Paget disease of the vulva (EMPV) is a rare skin disorder commonly seen in postmenopausal Caucasian females that appears clinically as red, eczematous, pruriginous, and sometimes painful lesions. Although most cases are noninvasive, EMPV may be associated with an underlying or distant adenocarcinoma. EMPV has a chronic and relapsing course. Read More

Cureus 2019 Oct 17;11(10):e5937. Epub 2019 Oct 17.

Dermatology, KCU-GME Consortium/Orlando Dermatology Program, Maitland, USA.

An 83-year-old Caucasian male presented with a pruritic erythematous patch to his right inguinal region, which he had contracted five months ago. After months of topical antifungal and steroid therapies, the patient was referred to dermatology due to a lack of lesion improvement. A 5. Read More

Gynecol Oncol 2020 Apr 25;157(1):146-150. Epub 2019 Nov 25.

Division of Gynecologic Oncology, Penn Medicine, Philadelphia, PA, USA.

Objective: To evaluate the clinicopathological characteristics, management and prognosis of patients with vulvar extramammary Paget disease of the vulva (EMPD).

Materials And Methods: The U.S National Cancer Database was accessed and patients diagnosed between 2004 and 2015 with microscopically confirmed vulvar EMPD were selected. Read More

Diagn Pathol 2019 Nov 7;14(1):125. Epub 2019 Nov 7.

Department of Pathology, Faculdade de Medicina FMUSP, Universidade de São Paulo, Av. Dr. Arnaldo, 455 - room 1149, Sao Paulo, SP, 01246-903, Brazil.

Background: Vulvar extramammary Paget disease is a rare chronic condition, that presents with non-specific symptoms such as pruritus and eczematous lesions. Because most of these lesions are noninvasive, the distinction between primary and secondary Paget disease is crucial to management.

Case Presentation: We report an unusual case of vulvar Paget disease associated with massive dermal vascular embolization, cervicovaginal involvement and metastasis to inguinal and retroperitoneal lymph nodes. Read More

Oncotarget 2019 Oct 22;10(58):6152-6167. Epub 2019 Oct 22.

Pathology, Memorial Sloan Kettering Cancer Center, New York, NY 10065, USA.

Extramammary Paget disease (EMPD) is a rare cutaneous adenocarcinoma of the anogenital region most commonly treated with surgical excision. Surgical margin clearance is often problematic and recurrence rates remain high indicating the need for additional therapeutic options. Topical immunomodulators have been used with reported success suggesting EMPD may respond to other immunotherapies. Read More

Cutis 2019 Sep;104(3):E26-E29

ProPath Dermatopathology, Dallas, and currently is retired, USA.

Indian J Dermatol Venereol Leprol 2020 Mar-Apr;86(2):134-140

Department of Urology, Huashan Hospital, Fudan University, Shanghai, China.

Background: Penoscrotal extramammary Paget's disease is a rare, slow-growing neoplasm with high frequency of local recurrence.

Aims: The aim of this study was to investigate the difference in clinicopathological characteristics between first-time and recurrent penoscrotal Paget's disease, and to discover the potential risk factors of recurrence.

Methods: Between January 2007 and February 2014, a total of 164 Chinese patients with biopsy-proven tramammary Paget's diseaseex in penis and scrotum underwent wide local resection in our institution. Read More

JAAD Case Rep 2019 Oct 25;5(10):868-870. Epub 2019 Sep 25.

Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.

Medicine (Baltimore) 2019 Oct;98(41):e17018

Gynecologic Oncology Unit, IRCCS Istituto Tumori "Giovanni Paolo II".

Paget's disease can arise in the breast (mammary Paget disease) or in other locations (extramammary Paget disease) such as anogenital skin in both males and females (Paget disease of the vulva [PDV]). Underlying adenocarcinoma can be found in some cases. This study aims to report clinical aspects, surgical procedures, outcomes, and recurrences of patients with PDV. Read More

Dis Colon Rectum 2019 11;62(11):1283-1293

Section of Colon and Rectal Surgery, Division of General and Gastrointestinal Surgery, Department of Surgery, General Hospital, Boston, Massachusetts.

Background: Extramammary Paget's disease is an uncommon intraepidermal adenocarcinoma with poorly defined clinical implications.

Objective: The purpose of this research was to estimate the risk of second primary neoplasms in patients with extramammary Paget's disease.

Design: This was a retrospective analysis of the Surveillance, Epidemiology, and End Results Registry (1973-2014). Read More

Intractable Rare Dis Res 2019 Aug;8(3):203-205

School of Podiatric Medicine, Barry University, Miami Shores, Florida, USA.

Extramammary Paget's disease (EMPD) is a carcinoma of the genital, perianal, and, rarely, axillary skin. The malignant Paget cells migrate extensively in the epidermis before invading the underlying dermis. Toker cells and keratinocytes have both been suggested as the cells of origin of EMPD. Read More

J Eur Acad Dermatol Venereol 2020 Apr 12;34(4):754-761. Epub 2019 Nov 12.

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

Background: The metastatic involvement of regional lymph nodes is the most important prognostic factor for overall survival of skin cancer patients. The sonographic technique of freehand real-time tissue elastography (RTE), which displays tissue rigidity as a colour overlay of the tissue image, was developed.

Objective: Our purpose was to evaluate the benefit of RTE for detecting lymph node metastases of skin cancer non-invasively before operation. Read More

J Urol 2019 12 13;202(6):1090. Epub 2019 Sep 13.

J Invest Dermatol 2020 Mar 9;140(3):583-592. Epub 2019 Sep 9.

Department of Dermatology, CHU of Sart Tilman, University of Liège, Liège, Belgium. Electronic address:

The molecular weight of methotrexate (MTX) makes cutaneous penetration difficult. Oxygen flow could enhance the skin permeation of MTX diluted in the proprietary LP3 carrier system. This pilot study aims to assess the efficacy, safety, and tolerance of oxygen flow-assisted LP3-MTX3% for treating superficial skin cancers. Read More

Clin Colon Rectal Surg 2019 Sep 22;32(5):386-393. Epub 2019 Aug 22.

Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio.

Anal cancer is a heterogeneous and rare disease process that accounts for ∼1.5% of all gastrointestinal tract malignancies. The perianal skin plays host to a variety of different premalignant and malignant lesions, all with variable histology, treatment options, and prognosis. Read More

J Dermatol 2019 Nov 9;46(11):e427-e429. Epub 2019 Sep 9.

Department of Dermatology, Hospital General Universitari de Castelló, Castelló de la Plana, Spain.

Hautarzt 2019 Sep;70(9):670-676

Klinik für Dermatologie und Venerologie, Vivantes Netzwerk für Gesundheit GmbH, Vivantes Klinikum Neukölln, Rudower Str. 48, 12351, Berlin, Deutschland.

Extramammary Paget's disease (EPD) is a rare, slowly growing, cutaneous adenocarcinoma with an incidence of 0.1-2.4 per 1,000,000 inhabitants. Read More

Br J Dermatol 2019 09;181(3):439-440

Institute of Cellular Medicine, Newcastle University, The Medical School, Framlington Place, Newcastle upon Tyne, NE2 4HH, U.K.

Br J Dermatol 2020 Feb 16;182(2):493-495. Epub 2019 Oct 16.

Department of Dermatology, University of Tsukuba, Tsukuba, Japan.

Pathol Int 2019 Oct 9;69(10):619-625. Epub 2019 Aug 9.

Department of Pathology, Tokyo Teishin University, Tokyo, Japan.

We report a case of cutaneous squamous cell carcinoma (CSCC), initially coexisting with invasive extramammary Paget's disease (EMPD), in the scrotum of an 84-year-old man. The patient initially had a rash and pruritus before presenting with a pedunculated scrotal mass surrounded by widespread erythema. He underwent total gastrectomy for adenocarcinoma 1 year previously and had been receiving TS-1 (Tegafur/Gimeracil/Oteracil pottasium) orally. Read More

Gastroenterology 2020 01 31;158(1):e5-e6. Epub 2019 Jul 31.

Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

Acta Medica (Hradec Kralove) 2019 ;62(2):77-81

2nd Academic Department of General Surgery, Aretaieion Hospital, National & Kapodistrian University of Athens, Greece.

Extramammary Paget disease (EMPD) is an uncommon intraepithelial malignancy, affecting the vulvo-perineal and perianal region, occurring in 6.5% of all Paget diseases. Usually, an underlying invasive adenocarcinoma denotes a more aggressive behaviour of the disease. Read More

Dermatol Surg 2020 Feb;46(2):151-158

Both authors are affiliated with the Department of Internal Medicine, Division of Dermatology, Washington University, St. Louis, Missouri.

Background: Extramammary Paget disease (EMPD) is a rare malignancy with unclear pathophysiology that occurs predominantly on apocrine rich skin. Surgery is the treatment of choice; however, procedures tend to be extensive and associated with a high rate of recurrence.

Objective: To review the current literature on EMPD regarding epidemiology, pathogenesis, clinical presentation, histology, diagnostic work-up, treatment, and prognosis. Read More

Front Med (Lausanne) 2019 10;6:160. Epub 2019 Jul 10.

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

Non-melanoma skin cancers (NMSCs), which represent a diverse group of cutaneous malignancies, are the most common forms of human neoplasia. The incidence of these diseases is increasing due to a number of factors, including that of increasing human lifespans. The majority of NMSCs are basal cell carcinomas (BCC) and cutaneous squamous cell carcinomas (cSCC), with the remainder being various rare skin cancers, including extramammary Paget's disease (EMPD), Merkel cell carcinoma (MCC), and several skin adnexal carcinomas. Read More

J Am Acad Dermatol 2019 Dec 23;81(6):1387-1396. Epub 2019 Jul 23.

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. Electronic address:

Vulvar malignancies represent a serious gynecologic health concern, especially given the increasing incidence over the past several decades. Squamous cell carcinoma and melanoma are common subtypes, although other neoplasms, such as basal cell carcinoma and Paget disease of the vulva, might be seen. Many vulvar cancers are initially misdiagnosed as inflammatory conditions, delaying diagnosis and worsening prognosis. Read More

Clin Nucl Med 2019 Oct;44(10):808-809

Oncology, Penang Adventist Hospital, George Town, Penang, Malaysia.

Extramammary Paget's disease (EMPD) is a rare disease with an estimated prevalence of 0.1 to 2.4 per 1,000,000 person-years. Read More

Am J Dermatopathol 2019 Aug;41(8):590-592

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

Australas J Dermatol 2020 Feb 18;61(1):e105-e107. Epub 2019 Jul 18.

Department of Dermatological Oncology, National Cancer Center Hospital, Chuo-ku.

J Cancer Res Clin Oncol 2019 Sep 11;145(9):2211-2225. Epub 2019 Jul 11.

Dipartimento Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Unità di Gineco-patologia e Patologia Mammaria, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Purpose: To investigate the expression of biological markers in primary vulvar Paget's disease (VPD).

Methods: Forty-one patients referred to a single major Center for Gynecologic Oncology from January 2008 to June 2018 were enrolled retrospectively: 30 non-invasive-VPD and 11 invasive-VPD. A total number of 60 samples, from all the 41 vulvar sites (VS), 8 metastatic lymph node sites (MLS) and 11 successive recurrent disease in vulvar site (RVS), were tested for an immunohistochemical panel, including the following markers: PD-L1, CD3, MSH2, MSH6, MLH1, PMS2, HER2/neu, EGFR, p16, p53, Ki67, ER, PR, AR, VEGF and CD31. Read More

Appl Immunohistochem Mol Morphol 2019 Jul 8. Epub 2019 Jul 8.

Departments of Pathology.

Extramammary Paget disease (EMPD) often involves apocrine gland-bearing locations including vulva and perianal area. EMPD of the scrotum is rare. Twenty patients were identified from the pathology files of 4 institutions between 2000 and 2018. Read More

Ned Tijdschr Geneeskd 2019 06 19;163. Epub 2019 Jun 19.

Diakonessenhuis, afd. Interne Geneeskunde, Utrecht.

A 72-year-old man was admitted to hospital because of backache. Physical examination also revealed a genital skin lesion with inguinal lymphadenopathy. Skin biopsy showed an infiltrating adenoma, arising from extramammary Paget disease. Read More

Australas J Dermatol 2019 Nov 21;60(4):e327-e329. Epub 2019 Jun 21.

Division of Dermatology, Department of Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

There has been a rising incidence of skin cancers among Asians in recent years. We present a retrospective analysis of 106 skin cancers and analysed the demography, clinical subtypes of skin cancers and surgical techniques used for skin cancer treatment. In our population, skin cancers were most frequently basal cell carcinomas and diagnosed among ethnic Chinese patients. Read More

Cochrane Database Syst Rev 2019 06 5;6:CD009245. Epub 2019 Jun 5.

Royal Devon and Exeter Hospital, Barrack Road, Exeter, UK, EX2 5DW.

Background: This is an updated version of the original Cochrane Review published in Issue 10, 2013.Extramammary Paget's disease is a rare form of superficial skin cancer. The most common site of involvement is the vulva. Read More

Cancers (Basel) 2019 May 29;11(6). Epub 2019 May 29.

Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.

Extramammary Paget disease (EMPD) is a locally aggressive cutaneous malignancy that usually arises in anogenital or axillary skin. Immune checkpoint inhibitors targeting programmed cell death receptor (PD-1) and/or its ligand (PD-L1) are approved for the treatment of several types of cancer, and response to these generally correlates with increased PD-L1 expression by tumor cells. The expression of PD-L1 and composition and density of the tumor-associated immune infiltrate in EMPD have been little studied. Read More

J Ultrasound Med 2019 Dec 29;38(12):3229-3237. Epub 2019 May 29.

Department of Medical Ultrasound, Shanghai Skin Disease Hospital, Ultrasound Research and Education Institute, Tongji University School of Medicine Shanghai, China.

Objectives: The purpose of this study was to investigate the performance of ultrasound biomicroscopy (UBM) and high-frequency ultrasound (HFUS) in the assessment of extramammary Paget disease (EMPD) and to correlate the imaging features with pathologic findings.

Methods: In this retrospective study, we described the imaging features from UBM and HFUS based on 17 pathologically proven EMPD cases. The performance for visualizing layer involvement by UBM and HFUS was compared. Read More

Ugeskr Laeger 2019 May;181(22)

Extramammary Pagets disease (EMPD) is a rare cancer and is often mistaken for benign dermatologic disorders such as eczema or psoriasis. The cancer is mostly primary but can be secondary to another cancer. EMPD is treated by surgical excision but a prolonged diagnostic process can have consequences for the patient due to spread of the primary tumour or growth and spread of the associated cancer. Read More

Clin Nucl Med 2019 Jul;44(7):e431-e432

Nuclear Medicine, Basavatarakam Indo American Cancer Hospital & Research Institute, Hyderabad, Telangana, India.

A 72-year-old man with history of itching for 8 months presented with an ill-defined, ulcerated, eczematous, thickened cutaneous lesion in the left perianal region. F-FDG PET/CT was performed, and neoplastic skin thickening with FDG uptake was seen at the left gluteal cleft. Hypermetabolic inguinal, retroperitoneal, and mediastinal lymph nodes, liver, and bone metastases were seen. Read More

Photodiagnosis Photodyn Ther 2019 Sep 14;27:92-94. Epub 2019 May 14.

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. Surgery is the standard treatment but relapse is common. The postoperative skin defects, penile reconstruction, functional effects and old age are also challenges for curing disease. Read More

Dermatol Clin 2019 Jul 19;37(3):261-267. Epub 2019 Apr 19.

Zitelli and Brodland PC, 5200 Centre Avenue, Suite 303, Pittsburgh, PA 15232, USA; Department of Dermatology, The University of Pittsburgh Medical Center, Pittsburgh, PA, USA; Department of Otolaryngology, The University of Pittsburgh Medical Center, Pittsburgh, PA, USA; Department of Plastic Surgery, The University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

Extramammary Paget disease is an intraepidermal adenocarcinoma, most often limited to the epidermis, with typical cases affecting genital skin. When limited to the epidermis, primary extramammary Paget disease is not life-threatening, but invasive disease may portend a poor prognosis. Surgical excision remains the mainstay of treatment of extramammary Paget disease, and Mohs micrographic surgery is the surgical treatment of choice. Read More

Medicine (Baltimore) 2019 May;98(19):e15243

Department of General Surgery, Xinqiao Hospital, Third Military Medical University, Chongqing, P.R. China.

Rational: Perianal Paget's disease (PPD) is a very rare intraepithelial adenocarcinoma. Very few cases of PPD have been reported till date, so the treatment remains controversial and more experience is needed.

Patient Concerns: A 73-year-old female was admitted to several hospitals with diagnosed as "perianal eczema" in perianal lesion. Read More

Int J Surg Pathol 2019 Oct 13;27(7):788-791. Epub 2019 May 13.

1 Icahn School of Medicine at Mount Sinai, New York, NY, USA.

We present a case of perianal goblet cell carcinoid with pagetoid spread. Goblet cell carcinoid, also known as adenocarcinoid tumor, predominantly arises as a primary appendiceal tumor and contains nests of neuroendocrine and mucin-containing cells. When this tumor type is seen in other sites it usually represents a metastasis. Read More