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Korean J Radiol 2020 Aug;21(8):955-966

Department of Radiology, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.

Ultrasound (US) is an attractive diagnostic approach to identify both common and uncommon nipple pathologies, such as duct ectasia, nipple abscess, nipple leiomyoma, nipple adenoma, fibroepithelial polyp, ductal carcinoma (restricted to nipple), invasive carcinoma, and Paget's disease. US is the reliable first-line imaging technique to assess nipple pathologies. It is useful to identify and characterize nipple lesions. Read More

Cureus 2020 Jun 12;12(6):e8586. Epub 2020 Jun 12.

Pathology and Laboratory Medicine/Histopathology, Aga Khan University Hospital, Karachi, PAK.

Nipple adenoma (NA) is a rare benign breast neoplasm that seldom co-exists with breast carcinoma (BC). Majority of these BC are separate from NA, and their origin from NA is an extremely rare event. We herein describe a case of 65-year-old female who had a painless lump for 15 years which increased in size and ulcerated for last six months. Read More

Pathol Int 2020 Jul 7. Epub 2020 Jul 7.

Department of Diagnostic Pathology, Oita University, Oita, Japan.

Pulmonary tumor thrombotic microangiopathy (PTTM) is histologically characterized by micro tumor cell embolism and intimal fibrocellular proliferation of pulmonary arteries or arterioles. We report a secondary case of PTTM associated with extramammary Paget's disease (EMPD). The patient was a 72-year-old man with exertional dyspnea. Read More

Dermatol Online J 2020 Mar 15;26(3). Epub 2020 Mar 15.

Department of Pathology, Englewood Hospital and Medical Center, Englewood, NJ.

Paget disease of the nipple is a rare presentation of breast cancer. It is sometimes mistaken for a benign skin condition of the nipple and surrounding areola, which can significantly delay the diagnosis and treatment. In over 90% of cases there is an underlying carcinoma, either ductal carcinoma in situ (DCIS) or invasive breast cancer. Read More

Skin Res Technol 2020 Jun 18. Epub 2020 Jun 18.

Department of Dermatology, Wuxi Second Affiliated Hospital of Nanjing Medical University, Wuxi, China.

Diagn Pathol 2020 Jun 11;15(1):75. Epub 2020 Jun 11.

Department of Pathology, Toho University School of Medicine, 5-21-16, Omori-Nishi, Ota-ku, Tokyo, 143-8540, Japan.

Background: We examined the vascularity of mammary Paget disease histologically to confirm the increased blood flow observed previously by clinical imaging. The relationships among blood vessel density (BVD), histopathological parameters of blood flow in the nipple, and the expression of angiogenic factors such as basic fibroblast growth factor (bFGF) and vascular endothelial growth factor A (VEGFA) were examined.

Methods: We calculated the average CD34-positive BVD and podoplanin (D2-40)-positive lymphatic vessel density (LVD) and the proportion of proliferating of endothelial cells in 14 Paget disease, 3 dermatitis biopsy, and 14 age-matched control cases. Read More

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

J Breast Cancer 2020 Apr 17;23(2):224-229. Epub 2020 Feb 17.

Department of Surgery, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.

We report a case of recurrence as Paget's disease at the core needle biopsy (CNB) entry site in a patient with microinvasive ductal carcinoma who underwent nipple-areola-skin sparing mastectomy (NASSM) and autologous reconstruction. Clinically diagnosed recurrences associated with previous needle procedures for malignant breast lesions are rare and usually occur in patients who have not received radiation therapy. The present case involved local recurrence at the skin puncture site of a patient diagnosed based on CNB findings who underwent NASSM without receiving radiation therapy. Read More

Breast Dis 2020 May 6. Epub 2020 May 6.

Division of Plastic Surgery, Mayo Clinic, Jacksonville, FL, USA.

Background: Paget's disease of the breast is rare. The National Cancer Database (NCDB) is one of the largest tumor databases in the United States.

Methods: We queried the NCDB to identify male and female patients with Paget's disease of the breast who were treated from January 1, 2004, through December 31, 2015. 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

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

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

Cancer Med 2020 Feb 3;9(4):1441-1450. Epub 2020 Jan 3.

Norris Comprehensive Cancer Center, University of Southern California, Los Angeles, CA, USA.

Background: Primary Extra-mammary Paget's disease (EMPD) is a very rare cutaneous adenocarcinoma affecting anogenital or axillary regions. It is characterized by a prolonged course with recurrences and eventually distant metastatic spread for which no specific therapy is known.

Methods: Eighteen EMPD (13 vulvar and five scrotal) and ten mammary Paget's disease (MPD) cases were comprehensively profiled for gene mutations, fusions and copy number alterations, and for therapy-relevant protein biomarkers). Read More

Ann R Coll Surg Engl 2020 Jan 6;102(1):62-66. Epub 2019 Sep 6.

Aberdeen Royal Infirmary, NHS Grampian, Aberdeen, UK.

Multifocal multicentric breast cancer has traditionally been considered a contraindication to breast conserving surgery because of concerns regarding locoregional control and risk of disease recurrence. However, the evidence supporting this practice is limited. Increasingly, many breast surgeons are advocating breast conservation in selected cases. Read More

J Dermatolog Treat 2019 Dec 27:1-3. Epub 2019 Dec 27.

Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

Extra-mammary Paget's disease is a rare form of intraepithelial adenocarcinoma with a variable pattern of invasion. Vulvar Paget's disease 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

Breast Cancer Res Treat 2020 Feb 13;179(3):577-584. Epub 2019 Nov 13.

Department of Medicine, Division of Hematology/Oncology, University of Miami, Miller School of Medicine, Miami, FL, 33136, USA.

Purpose: Paget's disease (PD) of the breast is an uncommon disease of the nipple usually accompanied by an underlying carcinoma, often HER2 + , and accounting for 0.5-5% of all breast cancer. To date, histogenesis of PD of the breast remains controversial, as two theories-transformation and epidermotropic-have been proposed to explain this disease. 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

Rev Colomb Obstet Ginecol 2019 03;70(1):58-67

Médica Ginecoobstetra; mastóloga. Docente titular, Universidad de Boyacá, Programa de Medicina. Grupo de investigación Hygea. Tunja (Colombia).

Objective: To present one case of Paget's disease (PD) of the breast without associated ductal carcinoma, and to conduct a review of the literature on its diagnosis and treatment.

Methods: 59-year-old woman with a two-year history of a chronic erythematous, ulcerated, itchy lesion in the left nipple, initially treated dermatologically with topical steroids with no improvement, finally visits a primary healthcare institution in the city of Tunja, Colombia for diagnosis. A biopsy and histochemical testing were performed to make the diagnosis and the lesion was then treated with central quadrantectomy. Read More

J Bone Miner Res 2019 12 14;34(12):2330. Epub 2019 Oct 14.

Department of Endocrinology and Internal Medicine, THG, Aarhus University Hospital, Aarhus, Denmark.

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

Int J Surg Pathol 2020 Apr 12;28(2):210-215. Epub 2019 Sep 12.

University of Texas Southwestern Medical Center, Dallas, TX, USA.

Mammary Paget's disease is rare and comprises about 0.62% of all breast cancer cases, only 1.65% of which occur in male patients. Read More

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

JAAD Case Rep 2019 Aug 5;5(8):709-712. Epub 2019 Aug 5.

University Hospitals Cleveland Medical Center, Cleveland, Ohio.

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

Eur J Surg Oncol 2019 Nov 26;45(11):2016-2021. Epub 2019 Jun 26.

The Nightingale Centre, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Southmoor Road, Manchester, M23 9LT, UK.

Introduction: Magseed is an alternative method of localising non-palpable breast lesions that has addressed many of the limitations of wire guided localisation (WGL). It consists of a paramagnetic seed that can be visualised on mammography and ultrasound. Intraoperative localisation of the seed is achieved with the use of the Sentimag probe. Read More

Lancet 2019 06 6;393(10191):2599-2612. Epub 2019 Jun 6.

Warwick Clinical Trials Unit, University of Warwick, Coventry, UK.

Background: Adjuvant trastuzumab significantly improves outcomes for patients with HER2-positive early breast cancer. The standard treatment duration is 12 months but shorter treatment could provide similar efficacy while reducing toxicities and cost. We aimed to investigate whether 6-month adjuvant trastuzumab treatment is non-inferior to the standard 12-month treatment regarding disease-free survival. Read More

Breast J 2019 07 6;25(4):706-708. Epub 2019 Jun 6.

Department of Anatomical Pathology, Singapore General Hospital, Singapore, Singapore.

Invasive ductal carcinoma of the nipple-areolar complex is exceedingly rare. Patients who present with bloody nipple discharge with or without the presence of Paget's disease constitute one-third of all symptomatic in situ patients. Only rarely does an invasive cancer cause nipple discharge in the absence of a clinical mass. 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

Cancer Med 2019 07 28;8(8):4043-4054. Epub 2019 May 28.

Department of Br, Surgery, People's Hospital of DeYang City, Deyang, China.

Background: Most mammary Paget disease (MPD) is associated with underlying in situ or invasive breast cancer. The objective of this study was to compare the clinicopathological characteristics and survival outcomes between breast cancer with Paget disease (PD) and breast cancer alone.

Methods: From the Surveillance, Epidemiology, and End Results (SEER) database, 2000-2015, of the US National Cancer Institute, we identified 1569 women who had PD with invasive ductal carcinoma (PD-IDC) and 1489 women who had PD with ductal carcinoma in situ (PD-DCIS). Read More

Dermatol Online J 2019 Apr 15;25(4). Epub 2019 Apr 15.

Veterans Affairs Medical Center Brooklyn, Brooklyn, New York SUNY Downstate, Department of Dermatology, Brooklyn, New York.

In 1874, Sir James Paget first described Paget disease of the nipple, also known as mammary Paget disease. In 1889, extramammary Paget disease (EMPD) of the scrotum and penis was identified. Although mammary and extramammary Paget disease are both characterized by epidermal Paget cells and share a similar clinical presentation, their uniqueness lies in anatomical location and histogenesis. Read More

J Surg Res 2019 09 24;241:178-187. Epub 2019 Apr 24.

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

Background: We aimed to analyze the association between Paget's disease (PD) and breast cancer (BC) subtypes and compare the effect of breast-conserving surgery (BCS) as a local treatment with mastectomy for PD.

Materials And Methods: Data of patients with histologic type International Classification of Diseases-0-3 8540-8543 who were treated from 1973 to 2014 were retrieved from the Surveillance, Epidemiology, and End Results database of the National Cancer Institute. A chi-square test was used to identify differences in categorical data among different groups. Read More

Exp Dermatol 2019 08 6;28(8):933-939. Epub 2019 Jun 6.

Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.

Malassezia yeast play a role in the pathogenesis of chronic dermatitis, especially in apocrine areas, by polarizing the local immunologic background to a Th2/Th17 state through aryl hydrocarbon receptor (AhR)-dependent pathways. Extra-mammary Paget's disease (EMPD) is an adenocarcinoma of apocrine origin, and except for cases associated with Malassezia yeast and their metabolites, the lesions typically develop in areas not exposed to environmental material. The purpose of this study was to investigate (a) the immunomodulatory effects of Malassezia metabolites on normal human keratinocytes (NHKCs), focusing on interleukin (IL)-17 and related cytokines/chemokines (IL-23, IL-36γ, CCL20), (b) the expression of these factors in lesion-affected skin in EMPD and (c) the activation of tumor-associated macrophages (TAMs) by these factors. Read More

Gan To Kagaku Ryoho 2019 Feb;46(2):348-350

Division of Breast and Endocrine Surgery, Dept. of Surgery, Nihon University School of Medicine.

The patient, a 41-year-old woman, presented with an elastic soft tumor on her left breast. Sore skin was observed on the left nipple and areola. Mammography revealed poor extension at the left nipple surrounding skin. Read More

J Invest Dermatol 2019 04 25;139(4):789-795. Epub 2018 Oct 25.

Department of Laboratory Medicine, First Affiliated Hospital of Shantou University Medical College, Shantou, Guangdong, China. Electronic address:

Paget's disease (PD) is an intraepidermal adenocarcinoma of the skin at the breast (mammary PD) or urogenital locations (extramammary PD [EMPD]). At present, there is lack of clarity on PD's pathogenesis, the relationship between its subtypes, and its lineage link with the underlying invasive carcinomas. Here we describe that mammary PD and EMPD have similar mutational profiles, with the most frequent recurrent mutations occurring in the chromatin remodeling genes, such as KMT2C (MLL3, 39%) and ARID2 (22%), with additional recurrent somatic mutations detected in genes previously not known to be mutated in cancers, such as CDCC168 (34%), FSIP2 (29%), CASP8AP2 (29%), and BIRC6 (24%). Read More

Oncologist 2019 06 7;24(6):e394-e396. Epub 2019 Mar 7.

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

Extramammary Paget's disease (EMPD) is a rare cutaneous adenocarcinoma that clinicopathologically resembles breast cancer. The prognosis of metastatic EMPD is poor. Although several chemotherapies have been tried, the effects are temporary; better drugs and combinations are required. Read More

Am J Dermatopathol 2019 Nov;41(11):867-868

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.

J Med Internet Res 2019 02 6;21(2):e12400. Epub 2019 Feb 6.

School of Health Sciences, Faculty of Health & Medical Sciences, University of Surrey, Guildford, United Kingdom.

Background: People engage in health information-seeking online when experiencing unusual or unfamiliar bodily changes. It is not well understood how people consult the internet for health information after the onset of unfamiliar symptoms and before receiving a potential diagnosis and how online information-seeking can help people appraise their symptoms. This lack of evidence may be partly due to methodological limitations in capturing in real time the online information-seeking process. 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.

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

Diagn Cytopathol 2019 Mar 28;47(3):249-250. Epub 2018 Nov 28.

Department of Pathology, Institut Curie, Paris, France.

J Am Acad Dermatol 2019 Jun 16;80(6):1467-1481. Epub 2018 Nov 16.

University of Connecticut Health Center Dermatology Department, Farmington, Connecticut. Electronic address:

The evaluation and management of dermatologic diseases of the breast and nipple requires an understanding of the unique anatomy of the breast and nipple and an awareness of the significant emotional, cultural, and sexual considerations that may come into play when treating this anatomic area. The first article in this continuing medical education series reviews breast anatomy, congenital breast anomalies, and benign and malignant breast tumors. An emphasis is placed on inflammatory breast cancer and breast cancer with noninflammatory skin involvement and on cutaneous metastases to the breast and from breast cancer. 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

Arch Pathol Lab Med 2019 02 20;143(2):206-211. Epub 2018 Aug 20.

From the Institute of Pathology (Drs D. L. Wachter, P. W. Wachter, Hartmann, and Strehl) and the Department of Gynecology and Obstetrics, Comprehensive Cancer Center Erlangen-European Metropolitan Area Nuremberg (Drs Fasching, Beckmann, and Hack), University Hospital, Erlangen, Germany; and OptiPath, Joint Practice for Pathology, Frankfurt am Main, Germany (Dr Riener).

Context.—: Paget disease of the breast, in most cases, represents intraepidermal spread of ductal carcinoma in situ. Molecular subtypes of invasive carcinoma of the breast have prognostic and therapeutic significance and show characteristic distribution. Read More

S D Med 2018 Aug;71(8):350-353

Department of Pathology, University of South Dakota Sanford School of Medicine, Sioux Falls, South Dakota.

Sebaceous carcinoma in situ is a poorly understood and ill-defined entity. In situ sebaceous carcinoma exhibits a similar location pattern to its invasive counterpart in that most commonly has a periorbital distribution. Review of the literature found only seven cases of extraocular sebaceous carcinoma in situ. Read More

Radiol Case Rep 2018 Jun 20;13(3):586-591. Epub 2018 Mar 20.

Department of Pathology, Sri Ramachandra University, Chennai, Tamil Nadu, India.

Mammary Paget disease is an uncommon malignancy of the breast that presents with ulceration or eczema of the nipple and is almost always associated with an underlying breast carcinoma. This disease is most commonly seen in the fifth and sixth decades of life and is almost always unilateral. The diagnosis of mammary Paget disease is generally based on clinical findings, confirmed by histopathologic examination. 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

Br J Dermatol 2018 12 9;179(6):1315-1321. Epub 2018 Sep 9.

Department of Obstetrics and Gynaecology (791), Radboud University Medical Center, 6500 HB, Nijmegen, the Netherlands.

Background: Vulvar Paget disease (VPD) is extremely rare and thought to be associated with other malignancies.

Objectives: To evaluate the risk of developing breast, intestinal and urological malignancies in patients with VPD compared with the general population, and in particular to focus on the risk of malignancy in patients with cutaneous noninvasive VPD.

Methods: Data on the oncological history of patients with any type of VPD between 2000 and 2015 were obtained from PALGA, a nationwide archive containing all pathology reports in the Netherlands. Read More

Int J Dermatol 2018 08 28;57(8):e49-e52. Epub 2018 May 28.

Faculty of Medicine, Department of Pathology, Ankara University, Ankara, Turkey.