1,750 results match your criteria Paget Disease Extramammary


Stop routine screening for associated malignancies in cutaneous non-invasive vulvar Paget disease?

Br J Dermatol 2018 Jun 19. Epub 2018 Jun 19.

Dept. Obstetrics & Gynaecology (791), Radboud university medical center, Nijmegen, the Netherlands.

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

Objective: The aim of this study was to evaluate the risk of developing breast, intestinal, and urological malignancies in patients with VPD compared with the general population, and in particular focus on the risk of malignancy in patients with cutaneous non-invasive VPD.

Methods: Data on the oncologic 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

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June 2018
1 Read

"Pigmented Extramammary Paget Disease"-A Potential Mimicker of Malignant Melanoma and a Pitfall in Diagnosis: A Case Report and Review of the Literature.

Am J Dermatopathol 2018 Jun 7. Epub 2018 Jun 7.

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

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June 2018
1 Read

Evidence-Based Screening Recommendations for Occult Cancers in the Setting of Newly Diagnosed Extramammary Paget Disease.

Mayo Clin Proc 2018 May 24. 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

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May 2018
4 Reads

Perianal extramammary Paget disease treated with topical imiquimod and oral cimetidine.

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

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April 2018
1 Read

Extramammary Paget disease of the penis closely mimicking the penile analogue of stratified mucin-producing intraepithelial lesion (SMILE).

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 -

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May 2018
1 Read

P16 Expression in Extramammary Paget's Disease of the Vulva and Scrotum Is Not Human Papillomavirus Related.

Int J Surg Pathol 2018 May 1:1066896918775513. Epub 2018 May 1.

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

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May 2018
1 Read

Imaging Features of Extramammary Paget Disease of the Scrotum: A Case Report.

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

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January 2018
4 Reads

Surgical Treatment for Extramammary Paget's Disease.

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

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May 2018
6 Reads

Docetaxel Combined With Cisplatin for Metastatic Extramammary Paget Disease.

Clin Genitourin Cancer 2018 Mar 23. 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

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March 2018
1 Read

Survival analysis of patients with invasive extramammary Paget disease: implications of anatomic sites.

BMC Cancer 2018 Apr 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

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April 2018
2 Reads

Transcriptome analyses reveal FOXA1 dysregulation in mammary and extramammary Paget's disease.

Hum Pathol 2018 Apr 6. Epub 2018 Apr 6.

Department of Pathology, Shantou University Medical College, Shantou, Guangdong; Department of Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, 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

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April 2018
2 Reads

Complete response of extramammary Paget's disease with imiquimod and PDT: Report of two cases.

Photodermatol Photoimmunol Photomed 2018 Mar 24. Epub 2018 Mar 24.

First Dermatology Department, Aristotle University of Thessaloniki, Thessaloniki, Greece.

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March 2018
1 Read

Genital diseases in the mature woman.

Clin Dermatol 2018 Mar - Apr;36(2):208-221. Epub 2017 Oct 3.

Department of Dermatology, Alpert Medical School of Brown University, Providence, RI; Department of Dermatology, Medical School of Jundiaí, São Paulo, Brazil. Electronic address:

Vulvovaginal conditions are common in mature women. This reflects age-related changes in immunity and skin barrier function of vulvovaginal tissues. Vaginal atrophy is commonly complicated by dryness and inflammation, which makes postmenopausal atrophic vaginitis a virtually ubiquitous condition. Read More

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October 2017
3 Reads

The Role of Radiotherapy in Extramammary Paget Disease: A Systematic Review.

Int J Gynecol Cancer 2018 May;28(4):829-839

Objective/purpose: Extramammary Paget disease (EMPD) is a rare neoplasm of the skin generally affecting the anogenital area. Because of the low-frequency of the disease, no specific guidelines about the treatment strategy are available. Surgery is the recommended therapy for resectable and localized disease, but several other local treatments have been reported such as radiotherapy (RT). Read More

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May 2018
2 Reads

Extramammary Paget disease with histologic presence of epidermolytic hyperkeratosis: An unusual presentation.

Indian J Dermatol Venereol Leprol 2018 May-Jun;84(3):341-344

Department of Dermatopathology, Skin Disease Hospital, Shanghai, Tongji University School of Medicine, Shanghai, China.

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March 2018
1 Read

Intraoperative Immunostaining for Cytokeratin-7 During Mohs Micrographic Surgery Demonstrates Low Local Recurrence Rates in Extramammary Paget's Disease.

Dermatol Surg 2018 Mar;44(3):354-364

Ali Hendi MD PC, Chevy Chase, Maryland.

Background: Extramammary Paget's disease (EMPD) is a rare intraepithelial malignancy with high recurrence rates following standard surgical treatments, ranging from 22% to 60% in large retrospective reviews.

Objective: To evaluate the local recurrence rate of Mohs micrographic surgery (MMS) supplemented with intraoperative immunohistochemistry for cytokeratin-7 (MMS + CK-7) for primary and recurrent EMPD.

Materials And Methods: Retrospective, multi-center, cross-sectional study of patients treated using MMS + CK-7. Read More

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March 2018
4 Reads

Primary vulvar Paget disease - the importance of clinical suspicion.

Dermatol Online J 2017 Oct 15;23(10). Epub 2017 Oct 15.

Dermatology Department, Centro Hospitalar do Porto, Portugal.

Extramammary Paget disease of the vulva is a rare condition that accounts for only 1-2% of vulvar malignancies and represents a frequent cause of misdiagnosis. It is most commonly seen in postmenopausal women. Clinically it is similar to Paget disease of the breast, appearing as red, well-demarcated eczematoid lesions, with slightly raised edges. Read More

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October 2017
4 Reads

Efficacy of low-dose 5-fluorouracil/cisplatin therapy for invasive extramammary Paget's disease.

J Dermatol 2018 May 14;45(5):560-563. Epub 2018 Feb 14.

Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.

Extramammary Paget's disease (EMPD) is one of the cutaneous adenocarcinomas. The effective chemotherapy for advanced EMPD has not been established. This study was designed to evaluate the efficacy of combination 5-fluorouracil (500 mg/body, 7 days/week) and cisplatin (5 mg/body 5 days/week) for invasive EMPD. Read More

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May 2018
4 Reads

Ectopic Extramammary Paget Disease Occurring on the Nose.

Dermatol Surg 2018 Feb 5. Epub 2018 Feb 5.

Department of Otolaryngology-Head and Neck Surgery, San Antonio Uniformed Services Health Education Consortium (SAUSHEC) San Antonio, Texas Department of Dermatology, Audie L. Murphy Memorial VA Hospital, San Antonio, Texas Department of Pathology, Audie L. Murphy Memorial VA Hospital, San Antonio, Texas Department of Otolaryngology-Head and Neck Surgery, San Antonio Uniformed Services Health Education Consortium (SAUSHEC) San Antonio, Texas.

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February 2018
2 Reads

[A Case Report of Adenocarcinoma with Pagetoid Growth(Secondary Paget's Disease)of the Anal Canal].

Gan To Kagaku Ryoho 2017 Nov;44(12):1332-1334

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

Differentiating primary Paget's disease from secondary Paget's disease, which is also known as Pagetoid growth of other cancers, in the perianal region is difficult. Given that the treatment strategies of both disease are significantly different, diagnosis of such conditions should be performed with caution. We report a case of secondary Paget's disease. Read More

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November 2017

Extramammary Paget disease of the vulva: immunohistochemical analysis of neoangiogenesis and epithelial-mesenchymal transition markers expression.

Diagn Pathol 2018 Jan 3;13(1). Epub 2018 Jan 3.

Gynecological Oncology Unit - CRO - IRCCS, National Cancer Institute, Aviano, Italy.

Background: Extra-mammary Paget's disease of the vulva (EMPDV) is an infrequent chronic disease that often recurs. The aim of the study was to assess the presence of neoangiogenesis and the expression of epithelial-mesenchymal transition (EMT) markers in EMPDV, and their potential correlation with stromal invasion.

Methods: All the women consecutively treated for EMPDV at our Institute, between January 2011 and December 2014, were studied for neoangiogenesis, analysed by microvessel density (MVD) using antibodies against CD31 and CD34. Read More

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January 2018
7 Reads

Extramammary Paget Disease of the Vulva: A Case Series Examining Treatment, Recurrence, and Malignant Transformation.

Int J Gynecol Cancer 2018 Mar;28(3):632-638

Objectives: Extramammary Paget disease (EMPD) of the vulva is a rare lesion with a high recurrence rate ranging from 12% to 61%. The rate of underlying adenocarcinoma varies, but in the largest series was reported at 4%. Given the rarity of the disease there is a paucity of data to optimize treatment. Read More

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March 2018
8 Reads

Fatal venous embolism in a patient with advanced extramammary Paget disease.

Eur J Dermatol 2017 12;27(6):647-648

Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University, Yonago, Japan.

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December 2017
3 Reads

Evaluation of mapping biopsies for extramammary Paget disease: A retrospective study.

J Am Acad Dermatol 2018 Jun 23;78(6):1171-1177.e4. Epub 2017 Dec 23.

Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

Background: Extramammary Paget disease (EMPD) sometimes shows an ill-defined border and an unexpectedly extended tumor spread beyond the clinical borders. Mapping biopsy is 1 approach for complete surgical removal, but its efficacy has remained controversial.

Objective: We sought to evaluate mapping biopsies for EMPD. Read More

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June 2018
1 Read

Perianal Paget disease: a report of 2 cases.

Ann Surg Treat Res 2017 Dec 1;93(6):336-341. Epub 2017 Dec 1.

Division of Colorectal Surgery, Department of Surgery, Colorectal Cancer Clinic, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.

Extramammary Paget disease (EMPD) is a rare cutaneous neoplasm. Perianal Paget disease (PPD) is a subset of EMPD manifesting perianal lesions. Two cases of PPD in Severance Hospital are described in this article. Read More

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December 2017
4 Reads

Coexistence of pemphigus herpetiformis with extramammary Paget disease.

Clin Exp Dermatol 2018 Apr 12;43(3):324-326. Epub 2017 Dec 12.

Department of Dermatology and Venereology, China-Japan Friendship Hospital, No. 2, Yinghua East Street, Chaoyang District, Beijing, 100029, China.

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April 2018
4 Reads

Reflectance confocal microscopy of mammary Paget disease.

Dermatol Pract Concept 2017 Oct 31;7(4):75-80. Epub 2017 Oct 31.

Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.

Mammary Paget disease is the intraepidermal adenocarcinoma of the nipple area which is characterized usually by a well-demarcated eczematous plaque. Reflectance confocal microscopy (RCM), is an in vivo noninvasive diagnostic tool with a high-resolution imaging of the skin, almost comparable to conventional histology. RCM findings of Paget disease are limited in the literature. Read More

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October 2017
3 Reads

Extramammary Paget's Disease Associated With Genital Wart and Lichen Sclerosus.

Acta Med Iran 2017 Sep;55(9):591-593

Department of Pathology, NYU Winthrop Hospital, Mineola, NY, USA.

Extramammary Paget's disease is an uncommon intraepithelial adenocarcinoma in genital and perianal regions. Genital wart is the most common sexually transmitted disease caused by human papilloma viruses and vulval lichen sclerosus is chronic pruritic dermatitis in genital area which could be able to change to invasive squamous cell carcinoma. We report a patient who had simultaneous lichen sclerosus, genital wart and extramammary Paget's disease of the vulva. Read More

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September 2017
3 Reads

Japan Society of Gynecologic Oncology guidelines 2015 for the treatment of vulvar cancer and vaginal cancer.

Int J Clin Oncol 2018 Apr 20;23(2):201-234. Epub 2017 Nov 20.

Department of Obstetrics and Gynecology, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.

Background: Vulvar cancer and vaginal cancer are relatively rare tumors, and there had been no established treatment principles or guidelines to treat these rare tumors in Japan. The first version of the Japan Society of Gynecologic Oncology (JSGO) guidelines for the treatment of vulvar cancer and vaginal cancer was published in 2015 in Japanese.

Objective: The JSGO committee decided to publish the English version of the JSGO guidelines worldwide, and hope it will be a useful guide to physicians in a similar situation as in Japan. Read More

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April 2018
1 Read

Imiquimod 5% cream as a therapeutic option for extramammary Paget's disease.

J Dermatol 2018 Feb 8;45(2):216-219. Epub 2017 Nov 8.

Department of Dermatology, Sapporo Medical University School of Medicine, Sapporo, Japan.

A wide local excision is the standard treatment for extramammary Paget's disease (EMPD), though this treatment often leads to permanent anogenital mutilation and functional impairment. The purpose of our study is to evaluate the efficacy and safety of the topical application of imiquimod 5% cream for non-invasive EMPD. We examined nine patients with EMPD. Read More

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February 2018
3 Reads

Mammary and Extramammary Paget's Disease Presented Different Expression Pattern of Steroid Hormone Receptors.

Biomed Res Int 2017 10;2017:3768247. Epub 2017 Sep 10.

Department of Laboratory Medicine, The First Affiliated Hospital of Shantou University Medical College, Shantou, Guangdong, China.

Background And Objectives: Paget's disease (PD) is a rare intraepithelial adenocarcinoma, which is composed of mammary (MPD) and extramammary Paget's disease (EMPD). Currently, the published literature contains scant data on expression pattern of steroid hormone receptors in MPD and EMPD.

Methods: Expression of estrogen receptor (ER) and androgen receptor (AR) was evaluated in 88 MPD and 72 EMPD by using immunohistochemical staining and H-score method. Read More

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Benefits of Urologic-Dermatologic Consultations for the Diagnosis of Cutaneous Penile Lesions: A Prospective Study.

Clin Genitourin Cancer 2018 Apr 5;16(2):e421-e424. Epub 2017 Oct 5.

Department of Urology, Angers University Hospital, Angers, France.

Introduction: We evaluated the benefits of a specialized consultation created in 2014 for cutaneous penile lesions.

Materials And Methods: We performed a descriptive prospective study evaluating all patients sent for a monthly urologic-dermatologic consultation at a French university hospital from September 2014 to September 2015 for cutaneous penile lesions. All patients evaluated were included. Read More

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April 2018
8 Reads

5-aminolevulinic acid (5-ALA) fluorescence-guided Mohs surgery resection of penile-scrotal extramammary Paget's disease.

Biosci Trends 2017 Nov 16;11(5):595-599. Epub 2017 Oct 16.

Department of Urology Research Institute, Huashan Hospital Affiliated to Fudan University.

This report aims to evaluate the usefulness of 5-aminolevulinic acid (5-ALA) fluorescence-guided Mohs surgery resection of penile-scrotal extramammary Paget's disease for achieving maximum tumor resection. Between January 2014 and December 2015, 5 patients underwent surgical resection of a penile-scrotal extramammary Paget's disease in department of urology, Huashan hospital, Fudan University. All patients were coated with 5-ALA (concentration of 20%) throughout the scrotum and the visible range of the lesion plus a 2cm margin 3 hours before the induction of anesthesia. Read More

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November 2017
1 Read

[A Case of Secondary Extramammary Paget's Disease Aroundthe Cutaneous Stoma after Radical Cystectomy].

Hinyokika Kiyo 2017 Sep;63(9):381-386

The Department of Urology, Osaka Red Cross Hospital.

We present a case of secondary extramammary Paget's disease around the cutaneous ureterostomy stoma after radical cystectomy. An 85-year-old man with bacillus calmette-guérin refractory high-grade urothelial carcinoma underwent radical cystectomy and cutaneous ureterostomy construction. After right ureter cancer diagnosis, he underwent right nephroureterectomy 3 years after the cystectomy. Read More

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September 2017
2 Reads

Invasive Paget Disease of the Vulva.

Int J Gynecol Cancer 2018 01;28(1):176-182

Objective: Extramammary Paget disease of the vulva (EPDV) is a rare occurrence with an indolent and relapsing course. Progression to invasion occurs in 4% to 19% of cases. The aim of this study is to report clinical-pathological features and outcomes of patients treated for invasive EPDV. Read More

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January 2018
4 Reads

Clinical Benefits of Preoperative Conventional Fluorescence Diagnosis in Surgical Treatment of Extramammary Paget Disease.

Dermatol Surg 2018 Mar;44(3):375-382

Department of Dermatology, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.

Background: In extramammary Paget disease (EMPD), initial margins of surgical excision are difficult to determine preoperatively. Fluorescence diagnosis (FD) may have utility as a noninvasive method for delineating tumor boundaries.

Objective: The relationship between FD-delineated borders and results of multiple scouting biopsies (MSBs) in EMPD was investigated, evaluating the potential clinical role of FD. Read More

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March 2018
5 Reads

A matter of margins: Surgical and pathologic risk factors for recurrence in extramammary Paget's disease.

Gynecol Oncol 2017 11 19;147(2):358-363. Epub 2017 Sep 19.

Mayo Clinic, Division of Gynecologic Oncology, United States.

Objectives: To determine surgical and pathologic variables associated with recurrence in extramammary Paget's disease (EMPD).

Methods: Medical records of patients seeking care for EMPD from 1/1992-9/2015 were reviewed. Follow-up was restricted to 5years following primary surgery. Read More

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November 2017
4 Reads

Chemokine Receptors CXCR4 and CXCR7 are Associated with Tumor Aggressiveness and Prognosis in Extramammary Paget Disease.

J Cancer 2017 2;8(13):2471-2477. Epub 2017 Aug 2.

Department of Urology, Fudan University Shanghai Cancer Center; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China.

Chemokines are involved in many aspects of oncogenesis, including regulation of cancer cell growth, dissemination and host-tumor response. However, the potential of the chemokine receptors, CXCR4 and CXCR7, in serving as biomarkers in extramammary Paget's disease (EMPD) has been rarely examined. Expressions of CXCR4 and CXCR7 were evaluated in 92 EMPD specimens by immunohistochemistry. Read More

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August 2017
6 Reads

Patients' Experiences With Extramammary Paget Disease: An Online Pilot Study Querying a Patient Support Group.

Urology 2018 Jan 8;111:214-219. Epub 2017 Sep 8.

Department of Urology, University of Washington Medical Center, Seattle, WA.

Objective: To illustrate the heterogeneous care delivered to patients with extramammary Paget disease (EMPD), a rare and lethal malignancy with poorly described treatment methodologies, by characterizing the clinical and pathologic characteristics of an international patient support group.

Materials And Methods: Institutional review board approval was obtained to develop and distribute a nonvalidated survey to patients from an international, online EMPD support group. The survey was developed to capture patient clinical and pathologic details and was distributed between January 2017 and February 2017. Read More

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January 2018
7 Reads

Successful TS-1 monotherapy as the second-line treatment for advanced extramammary Paget's disease: A report of two cases.

J Dermatol 2018 Jan 11;45(1):80-82. Epub 2017 Sep 11.

Department of Dermatology, Sapporo Medical University School of Medicine, Sapporo, Japan.

There is no standard chemotherapeutic treatment for advanced extramammary Paget's disease, though the effectiveness of some chemotherapy regimens, including docetaxel, has been reported. In this report, we report that TS-1 monotherapy was effective in two patients with advanced extramammary Paget's disease after docetaxel treatment failure. TS-1 monotherapy may be useful as the second-line treatment for patients with advanced extramammary Paget's disease. Read More

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January 2018
4 Reads

A rare case of metastatic extramammary Paget disease of the spine and review of the literature.

J Clin Neurosci 2017 Nov 6;45:161-165. Epub 2017 Sep 6.

Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address:

Extramammary Paget disease is an intraepithelial neoplasm affecting cells rich in apocrine glands-often located in the vulvar, scrotal, or perianal region. It typically affects older patients, between the ages of 50 and 80years old, and is most often limited to the epidermis. A 47-year-old Asian male first presented with enlargement of the right inguinal lymph node. Read More

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November 2017
3 Reads

The Paget Trial: A Multicenter, Observational Cohort Intervention Study for the Clinical Efficacy, Safety, and Immunological Response of Topical 5% Imiquimod Cream for Vulvar Paget Disease.

JMIR Res Protoc 2017 Sep 6;6(9):e178. Epub 2017 Sep 6.

Department of Obstetrics and Gynaecology, Radboud University Medical Center, Nijmegen, Netherlands.

Background: Vulvar Paget disease is a rare skin disorder, which is most common in postmenopausal Caucasian women. They usually present with an erythematous plaque that may show fine or typical "cake icing" scaling or ulceration that may cause itching, pain, irritation, or a burning sensation. Although most cases are noninvasive, vulvar Paget disease may be invasive or associated with an underlying vulvar or distant adenocarcinoma. Read More

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September 2017
7 Reads

New technology discloses the tumour margin of extramammary Paget disease?

Authors:
N Hatta

Br J Dermatol 2017 07;177(1):18-19

Department of Dermatology, Toyama Prefectural Central Hospital, 2-2-78 Nishinagae, Toyama, 930-8550, Japan.

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July 2017
1 Read

GATA3 is a sensitive marker for primary genital extramammary paget disease: an immunohistochemical study of 72 cases with comparison to gross cystic disease fluid protein 15.

Diagn Pathol 2017 Jul 10;12(1):51. Epub 2017 Jul 10.

Department of Pathology and Immunology, Washington University School of Medicine, 660 S South Euclid Avenue Campus Box 8118, Saint Louis, MO, 63110, USA.

Background: GATA-binding protein 3 (GATA3) has been identified as a sensitive marker for breast carcinoma but its sensitivity in primary genital extramammary Paget diseases (EMPDs) has not been well studied.

Methods: Here we investigated immunohistochemical expression of GATA3 in 72 primary genital EMPDs (35 from female, 37 from male; 45 with intraepithelial disease only, 26 with both intraepithelial disease and invasive adenocarcinoma including 14 also metastasis, 1 with metastatic adenocarcinoma only for study). We also compared GATA3 to gross cystic disease fluid protein 15 (GCDFP15) for their sensitivity. Read More

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July 2017
35 Reads

Small Intestinal Submucosal Matrix as a Novel Reconstructive Option for Large Scrotal Defects.

Dermatol Surg 2018 Feb;44(2):318-321

Department of Dermatology, Indiana University School of Medicine, Indianapolis, Indiana.

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February 2018

[Anogenital mammary-like glands and related diseases. Part 2. Malignant tumors of the anogenital glands].

Arkh Patol 2017;79(3):47-52

Department of Pathology, Charles University, Plzen, Czech Republic.

Mammary-like glands are a normal anatomical component of the anogenital region and can give rise to many benign and malignant tumors that morphologically mimic the similar diseases of the breast. The literature review is complemented by a description of 199 cases of malignant tumors of mammary-like glands. The paper presents the clinical and morphological characteristics of various malignant tumors of mammary-like glands, including extramammary Paget's disease, ductal, tubulolobular, adenoid cystic adenocarcinoma, low-grade phyllodes tumor, etc. Read More

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Spectrum of Changes in Anogenital Mammary-like Glands in Primary Extramammary (Anogenital) Paget Disease and Their Possible Role in the Pathogenesis of the Disease.

Am J Surg Pathol 2017 Aug;41(8):1053-1058

*Department of Pathology, Clinical Research and Practical Center for Specialized Oncological Care †Department of Pathology, Medical Faculty, Saint-Petersburg State University ‡Department of Pathology, Saint-Petersburg Medico-Social Institute ∥∥Department of Dermatology, Medical Military Academy, Saint Petersburg, Russia §PathWest Laboratory Medicine WA, QEII Medical Centre ∥School of Pathology and Laboratory Medicine, University of Western Australia, Nedlands ¶PathWest Laboratory Medicine WA, King Edward Memorial Hospital, Perth, WA, Australia #Sikl's Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague **Bioptical Laboratory §§Department of Obstetrics and Gynecology, Charles University Medical Faculty Hospital, Pilsen, Czech Republic ††Miraca Life Science, Irving, TX ‡‡Department of Pathology, Division of Dermatopathology, Medical College of Wisconsin, Milwaukee, WI ¶¶Dermatopathology Unit, Department of Dermatology, Zurich University Hospital, Zurich, Switzerland.

To determine whether a subset of primary extramammary Paget disease (EMPD) may originate in anogenital mammary-like glands (AGMLG), the authors studied 181 specimens of EMPD, detailing alterations in AGMLG. The latter were identified in 33 specimens from 31 patients. All patients were women, ranging in age from 38 to 93 years (median, 65 y). Read More

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August 2017
17 Reads

Perianal Pagetoid Intraepithelial Carcinoma.

Case Rep Gastroenterol 2017 Jan-Apr;11(1):109-113. Epub 2017 Mar 3.

aDivision of Radiation Oncology, The Ottawa Hospital and the University of Ottawa, Ottawa, Ontario, Canada.

Extramammary Paget disease (EMPD) involving the perianal region is rare and challenging to manage. Primary EMPD involves stand-alone noninvasive lesions within the epidermis, while secondary EMPD involves phenotypically similar lesions derived from separate underlying malignancies. Differentiating between primary and secondary EMPD is challenging when no underlying malignancies are detected during workup. Read More

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March 2017
6 Reads

Extramammary Paget's disease of the oral mucosa and perioral skin.

Oral Surg Oral Med Oral Pathol Oral Radiol 2017 Aug 11;124(2):e157-e163. Epub 2017 May 11.

State Key Laboratory of Oral Diseases, Department of Oral Medicine, West China Hospital of Stomatology, Sichuan University, Chengdu, Sichuan, P.R. China. Electronic address:

Extramammary Paget's disease (EMPD) is an unusual intraepidermal adenocarcinoma. Only 2 cases of EMPD in the oral mucosa have been reported previously. Here, we present the first report of a rare case of Paget's disease of the oral mucosa and perioral skin with extensive contiguous erythematous and erosive manifestations. Read More

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August 2017
2 Reads