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    1717 results match your criteria Paget Disease Extramammary

    1 OF 35

    Extramammary Paget Disease of the Vulva: A Case Series Examining Treatment, Recurrence, and Malignant Transformation.
    Int J Gynecol Cancer 2018 Jan 12. Epub 2018 Jan 12.
    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

    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

    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

    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

    Imiquimod 5% cream as a therapeutic option for extramammary Paget's disease.
    J Dermatol 2017 Nov 8. 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

    Benefits of Urologic-Dermatologic Consultations for the Diagnosis of Cutaneous Penile Lesions: A Prospective Study.
    Clin Genitourin Cancer 2017 Oct 5. 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

    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

    Invasive Paget Disease of the Vulva.
    Int J Gynecol Cancer 2018 Jan;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

    Clinical Benefits of Preoperative Conventional Fluorescence Diagnosis in Surgical Treatment of Extramammary Paget Disease.
    Dermatol Surg 2017 Sep 20. Epub 2017 Sep 20.
    *Department of Dermatology, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China; †Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, 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

    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

    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

    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

    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

    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

    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

    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

    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

    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

    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

    Intratumor dihydropyrimidine dehydrogenase mRNA expression levels are decreased in extramammary Paget's disease.
    Drug Discov Ther 2017 Jul 11;11(3):152-155. Epub 2017 Jul 11.
    Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University.
    S-1, a 5-fluorouracil (5-FU)-based anti-cancer agent, is an important drug for treating metastatic extramammary Paget's disease (EMPD). Although intratumor expression levels of 5-FU metabolism enzymes have been studied widely in many solid tumors, no studies have examined on the expression levels of thymidylate synthase (TS), orotate phosphoribosyl-transferase (OPRT) or dihydropyrimidine dehydrogenase (DPD) in skin cancers. The aim of this study was to estimate the intratumoral mRNA expression levels of these genes in EMPD by real time PCR. Read More

    Invasive Extramammary Paget Disease of the Vulva With Signet Ring Cell Morphology in a Patient With Signet Ring Cell Carcinoma of the Stomach: Report of a Case.
    Int J Gynecol Pathol 2017 Jun 2. Epub 2017 Jun 2.
    Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
    This report describes a rare case of invasive extramammary Paget disease of the vulva with signet ring cell morphology in a 58-yr-old woman with a history of signet ring cell carcinoma of the stomach. This case was initially misinterpreted as a metastatic gastric carcinoma to the vulva because an initial small, superficial biopsy specimen showed infiltration of signet ring cells in the dermis without intraepidermal Paget cells. However, a surgically resected specimen showed concordant immunophenotypes in both intraepidermal Paget cells and intradermal signet ring cell components with immunoreactivity to cytokeratin (CK) 7, CEA, and gross cystic disease fluid protein-15, and immunonegativity for CK20, MUC5AC, and MUC6. Read More

    Diagnostic Criteria in Intraepithelial Pagetoid Neoplasms: A Histopathologic Study and Evaluation of Select Features in Paget Disease, Bowen Disease, and Melanoma In Situ.
    Am J Dermatopathol 2017 Jun;39(6):419-427
    *Demonstrator at the Dermatology Department, Kasralainy Faculty of Medicine, Cairo, University, Egypt; †Visiting Research Fellow, Ackerman Academy of Dermatopathology, New York, NY; ‡Attending Dermatologist, Department of Dermatology, Dermatology Hospital of Southern Medical University, Guangdong Provincial Dermatology Hospital, China; §Junior Specialist, Department of Dermatology, University of California, Irvine, CA; ¶Attending Dermatologist, Department of Dermatology, Jose R. Reyes Memorial Medical Center, Manila; ‖Medical Student, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ; #Professor of Biostatistics, Department of Biostatistics and Epidemiology, National Cancer Institute, Cairo University, Cairo, Egypt; **Dermatopathologist, Dermpath Diagnostics New York, Port Chester, NY; and ††Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, SC.
    Background: Paget disease, Bowen disease, and malignant melanoma in situ are intraepidermal neoplasms, characterized by the presence of pagetoid scatter of atypical cells in the epidermis. This study reviewed the frequency of select histologic criteria to validate their usefulness in the histologic distinction between these entities.

    Methods: One hundred forty-four specimens with the diagnosis of Bowen disease, 144 specimens with Paget disease (mammary and extramammary), and 144 specimens with malignant melanoma in situ were examined microscopically to define frequencies of select histologic criteria present in each disease. Read More

    Handheld Reflectance Confocal Microscopy for the Detection of Recurrent Extramammary Paget Disease.
    JAMA Dermatol 2017 Jul;153(7):689-693
    Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.
    Importance: Extramammary Paget disease (EMPD) is commonly refractory to surgical and nonsurgical therapies. Identifying recurrent or persistent EMPD is challenging because the disease is multifocal, and multiple blind scouting biopsies are usually performed in this setting. Handheld reflectance confocal microscopy (HRCM) has been used to diagnose and map primary EMPD and therefore may be used to identify EMPD recurrences. Read More

    A case of extramammary inguinal Paget disease in a male patient: surgical treatment with an abdominal advancement cutaneous flap.
    Acta Biomed 2017 Apr 28;88(1):79-81. Epub 2017 Apr 28.
    Extramammary Paget disease (EMPD) is a rare neoplasm. The clinical case of a 55-year-old man with a two-year history of a pruritic, painless erythematous skin rash on the inguinal region and scrotum is described. After a delay due to improper diagnosis and improper treatments, the patient came to the attention of the Division of plastic surgery. Read More

    Multiple concurrent primary extramammary Paget's disease.
    Malays Fam Physician 2016 30;11(1):18-21. Epub 2016 Apr 30.
    Department of Family Medicine, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Center Kuala Lumpur, Malaysia.
    Extramammary Paget's disease (EMPD) is a rare malignant disorder of the skin, which was described in as early as the nineteenth century. EMPD usually occurs as a single lesion in the apocrine sweat gland-bearing skin with abundant hair follicles. Here, we present an elderly man who suffered from a non-resolving chronic genital pruritus for 8 months. Read More

    Cervical metastases originating from a primary rectal adenocarcinoma due to a pagetoid spread.
    Hum Pathol 2017 10 28;68:184-188. Epub 2017 Apr 28.
    Department of Pathology, Radboud University Medical Center, 6500 HB, Nijmegen, the Netherlands.
    Vulvar Paget disease is a rare skin disorder, considered an in situ adenocarcinoma. It is characterized by intraepithelial Paget cells, of which the origin is unclear. About 75% of cases have a cutaneous origin; the other 25% originate from an intestinal or urological malignancy. Read More

    Paget's Disease of the Vulva: A Review of 20 Years' Experience.
    Int J Gynecol Cancer 2017 May;27(4):791-793
    *Department of Gyn Oncology, Hereford Hospital, Hereford; †Department of Gyn Oncology, University of Bristol, Bristol; and ‡Department of Gyn Oncology, St James's Institute of Oncology, Leeds, United Kingdom.
    Background: Extramammary Paget's disease is a rare condition, and the vulva is a common site for it to occur. Despite this, there is a paucity of literature on Paget's disease of the vulva (VPD). A Cochrane meta-analysis could not draw any conclusions on interventions in VPD. Read More

    Molecular subtyping of mammary-like adenocarcinoma of the vulva shows molecular similarity to breast carcinomas.
    Histopathology 2017 Sep 16;71(3):446-452. Epub 2017 Jun 16.
    Division of Anatomical Pathology, Vancouver General Hospital, University of British Columbia, Vancouver, Canada.
    Aims: Mammary-like adenocarcinoma (MLA) of the vulva is thought to be derived from vulvar mammary-like glands. The aim of this study was to characterize a series of MLAs by using an immunohistochemical algorithm that identifies the major molecular subtypes of breast cancer.

    Methods And Results: Seven cases of vulval MLA were stained for oestrogen receptor (ER), progesterone receptor (PR), human epidermal growth factor receptor 2 (HER2), Ki67, epidermal growth factor receptor (EGFR), cytokeratin (CK) 5, nestin, and inositol polyphosphate-4-phosphatase (INPP4b). Read More

    Modified weekly regimen of cisplatin, epirubicin and paclitaxel induced a durable response in two cases of metastatic extramammary Paget's disease.
    J Dermatol 2017 Oct 13;44(10):1148-1151. Epub 2017 Apr 13.
    Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
    Metastatic extramammary Paget's disease (EMPD) is a rare cancer with no standardized treatment. We report two cases of metastatic EMPD treated with a modified weekly PET (cisplatin, epirubicin and paclitaxel) regimen given biweekly (i.e. Read More

    Vulvar and Anal Intraepithelial Neoplasia: Terminology, Diagnosis, and Ancillary Studies.
    Adv Anat Pathol 2017 May;24(3):136-150
    Department of Pathology, Stanford University School of Medicine, Stanford, CA.
    Currently, it is recognized that there is an HPV-related and an HPV-independent pathway to developing squamous cell carcinomas (SCC) in the anus and vulva. The majority of precursor lesions and SCC in the anus and vulva are high-risk HPV-associated, with HPV16 the most common type. Given the morphologic overlap and biological equivalence of HPV-related preinvasive squamous lesions of the lower anogenital tract, a unified, 2-tiered histopathologic nomenclature is now recommended. Read More

    Podoplanin expression in peritumoral keratinocytes predicts aggressive behavior in extramammary Paget's disease.
    J Dermatol Sci 2017 Jul 27;87(1):29-35. Epub 2017 Mar 27.
    Department of Dermatology, Kyoto Prefectural University of Medicine, Kyoto, Japan. Electronic address:
    Background: Recent studies have demonstrated podoplanin expression in several tumors, which has been associated with lymph node metastasis and poor prognosis. Podoplanin expression in peritumoral cells such as cancer-associated fibroblasts also correlates with tumor progression in several cancers. However, podoplanin expression and its association with extramammary Paget's disease (EMPD) remain unclear. Read More

    Evaluation of positron emission tomography imaging to detect lymph node metastases in patients with extramammary Paget's disease.
    J Dermatol 2017 Aug 11;44(8):939-943. Epub 2017 Mar 11.
    Department of Dermatology, Hamamatsu University School of Medicine, Hamamatsu, Japan.
    Patients with extramammary Paget's disease (EMPD) have a relatively good prognosis, when spread of the tumor cells is limited to the epidermis. However, invasive EMPD has a poor prognosis, when the patients have regional lymph node metastasis. Detection of nodal metastasis is thus mandatory to manage EMPD. Read More

    Study of Selected BRCA1, BRCA2, and PIK3CA Mutations in Benign and Malignant Lesions of Anogenital Mammary-Like Glands.
    Am J Dermatopathol 2017 May;39(5):358-362
    *Department of Pathology, Clinical Research and Practical Center for Specialized Oncological Care, Saint Petersburg, Russia; †Department of Pathology, Medical Faculty, Saint-Petersburg State University, Saint-Petersburg, Russia; ‡Department of Pathology, Saint-Petersburg Medico-Social Institute, Saint Petersburg, Russia; Departments of §Pathology, and ¶Tumor Growth Biology, Petrov's Research Institute of Oncology, Saint Petersburg, Russia; ‖Department of Medical Genetics, Saint Petersburg Pediatric Medical University, Saint Petersburg, Russia; **Sikl's Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague, Pilsen, Czech Republic; and ††Bioptical Laboratory, Pilsen, Czech Republic.
    Anogenital mammary-like glands (AGMLGs) are nowadays considered a normal component of the anogenital area. Lesions involving AGMLGs are histopathologically very similar to their mammary counterparts, but the information on molecular biological mechanisms in these vulvar/perianal tumors is scarce. Mutations in the PI3K-AKT cascade have been found in hidradenoma papilliferum. Read More

    [Clinicopathological study of 56 cases of extramammary Paget's disease with or without acantholysis].
    Zhonghua Yi Xue Za Zhi 2017 Feb;97(8):598-602
    Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
    Objective: To identify the clinicopathological features of extramammary Paget's disease(EMPD) and investigate the clinical and histopathological significance of acantholysis in EMPD. Methods: From June, 2010 to October, 2016, a total of 56 patients were diagnosed as EMPD in the Department of Dermatology, Peking Union Medical College Hospital. Clinical and histopathological data were retrieved from these patients' medical records and analyzed respectively. Read More

    Paget's disease of the anus masking a mixed adenoneuroendocrine tumour of the rectum.
    BMJ Case Rep 2017 Feb 14;2017. Epub 2017 Feb 14.
    Department of General Surgery, NYU Lutheran, Brooklyn, New York, USA.
    A man aged 83 years with vague perirectal symptoms had a delayed diagnosis of Paget's disease of the anus. A lack of thorough digital rectal examination failed to diagnose a mixed adenonueroendocrine tumour of the rectum in a timely matter. Read More

    Surgical Treatment and Outcomes of Patients With Extramammary Paget Disease: A Cohort Study.
    Dermatol Surg 2017 May;43(5):708-714
    *Mayo Medical School, Mayo Clinic College of Medicine, Rochester, Minnesota; †Department of Dermatology, Mayo Clinic, Rochester, Minnesota; ‡Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota.
    Background: Extramammary Paget disease (EMPD) is a rare intraepithelial adenocarcinoma occurring mainly in the anogenital region. Traditional management with wide local excision has shown high recurrence rates, thus Mohs micrographic surgery (MMS) has emerged as a promising treatment option.

    Objective: To compare long-term outcomes after treatment with MMS or excision for primary EMPD. Read More

    Successful use of radiotherapy in the treatment of extramammary Paget's disease in vulvar area.
    G Ital Dermatol Venereol 2017 Apr;152(2):181-182
    Dermatology Unit, Department of Neurosciences, Mental Health and Sense Organs (NESMOS), Sant'Andrea Hospital, Faculty of Medicine and Psychology, "Sapienza" University, Rome, Italy.

    Serum cytokeratin 19 fragment 21-1 is a useful tumor marker for the assessment of extramammary Paget's disease.
    J Dermatol 2017 Jun 2;44(6):666-670. Epub 2017 Feb 2.
    Department of Dermatology, Sapporo Medical University School of Medicine, Sapporo, Japan.
    Cytokeratin 19 fragment 21-1 (CYFRA 21-1) has been used as a tumor marker for several malignancies. However, to date, no studies have assessed whether CYFRA 21-1 could be a useful marker for extramammary Paget's disease (EMPD). The present study aimed to evaluate the significance of CYFRA 21-1 as a serum tumor marker for EMPD progression. Read More

    Three-dimensional evaluation of subclinical extension of extramammary Paget disease: visualization of the histological border and its comparison to the clinical border.
    Br J Dermatol 2017 Jul 4;177(1):229-237. Epub 2017 May 4.
    Department of Dermatology, Kyoto University Graduate School of Medicine, 54 Shogoin-Kawahara, Sakyo-ku, Kyoto, 606-8507, Japan.
    Background: In extramammary Paget disease (EMPD), Paget cells are sometimes detected outside the clinical border (subclinical extension). However, the spreading pattern of Paget cells in subclinical extension remains unclear. In addition, the macroscopic appearances of lesions accompanied by subclinical extension are totally unknown. Read More

    Mayo Clinic Cancer Center Experience of Metastatic Extramammary Paget Disease 1998-2012.
    Rare Tumors 2016 Nov 17;8(4):6804. Epub 2016 Nov 17.
    Hematology Oncology Department, Mayo Clinic Rochester , MN, USA.
    Extramammary Paget disease (EMPD) is a rare cutaneous malignancy. The most common presentation of EMPD is the vulva followed by perianal involvement. Most cases are localized to the dermis with treatment focused on surgery, topical treatment or radiotherapy. Read More

    Extramammary Paget Disease of the Vulva - Case Report.
    Rev Bras Ginecol Obstet 2016 Oct 10;38(10):524-528. Epub 2016 Nov 10.
    Department of Gynecology and Obstetrics, Health Sciences Center, Universidade Federal de Santa Catarina, Florianópolis, SC, Brazil.
    Extramammary Paget disease (EPD) is a rare malign neoplasm that may affect the vulva and has manifestations common to benign diseases such as itching, pain and eczema. This leads to delay in diagnosis and consequent worse prognosis. The definitive diagnosis is obtained by biopsy of the vulva, which shows Paget cells. Read More

    Chemotherapy reaction induced by ixabepilone, a microtubule stabilizing agent, mimicking extramammary Paget's disease in a patient with breast carcinoma.
    J Cutan Pathol 2016 Dec 24;43(12):1215-1219. Epub 2016 Oct 24.
    Department of Dermatology, University of California, Davis, CA, USA.
    The histopathologic characteristics of reactions caused by the many novel anticancer agents are under-recognized. We report a case of a 67-year-old female with locally advanced metastatic breast cancer, who initially presented with an extensive reticulated erythematous patch on the trunk caused by intravascular metastases confirmed by a skin biopsy. Owing to disease progression, she was started on ixabepilone, a mitotic inhibitor. Read More

    Spinal Metastases of Extramammary Paget Disease with Radiologic-Pathologic Correlation.
    J Radiol Case Rep 2016 May 31;10(5):1-8. Epub 2016 May 31.
    Department of Radiology, Mayo Clinic, Rochester, MN, USA.
    Extramammary Paget disease (EMPD) is an uncommon malignancy. It manifests either in the primary form in the skin as an intraepithelial neoplasm, or in secondary form as pagetoid (intraepithelial) spread of an underlying internal carcinoma to the skin. Although local invasion and recurrence of primary extramammary Paget disease are relatively frequent, widespread metastases are rare. Read More

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