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    1655 results match your criteria Paget Disease Extramammary

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    Surgical Treatment and Outcomes of Patients With Extramammary Paget Disease: A Cohort Study.
    Dermatol Surg 2017 Feb 6. Epub 2017 Feb 6.
    *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

    Serum cytokeratin 19 fragment 21-1 is a useful tumor marker for the assessment of extramammary Paget's disease.
    J Dermatol 2017 Feb 2. 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's disease: Visualization of histological border and its comparison to clinical border.
    Br J Dermatol 2016 Dec 27. Epub 2016 Dec 27.
    Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, 606-8507, Japan.
    Background: In extramammary Paget's disease (EMPD), Paget cells are sometimes detected outside the clinical border (subclinical extension). The spreading pattern of Paget cells in subclinical extension, however, 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

    Depth and Patterns of Adnexal Involvement in Primary Extramammary (Anogenital) Paget Disease: A Study of 178 Lesions From 146 Patients.
    Am J Dermatopathol 2016 Nov;38(11):802-808
    *Department of Pathology, Clinical Research and Practical Center for Specialized Oncological Care, Saint-Petersburg, Russia; †Department of Pathology, Medical Faculty, Saint-Petersburg State University, Russia; ‡Department of Pathology, Petrov's Research Institute of Oncology, Saint Petersburg, Russia; §PathWest Laboratory Medicine WA, King Edward Memorial Hospital, Perth, Western Australia, Australia; ¶PathWest Laboratory Medicine WA, QEII Medical Centre, Nedlands, WA, Australia; ‖University of Western Australia, School of Pathology and Laboratory Medicine, Nedlands, WA, Australia; **Dermatopathologische Gemeinschaftspraxis, Friedrichshafen, Germany; ††Sikl's Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague, Pilsen, Czech Republic; ‡‡Bioptical Laboratory, Pilsen, Czech Republic; §§Miraca Life Science, Irving, TX; ¶¶Department of Pathology, Division of Dermatopathology, Medical College of Wisconsin, Milwaukee, WI; ‖‖Department of Obstetrics and Gynecology, Charles University Medical Faculty Hospital, Pilsen, Czech Republic; ***Department of Pathology, Regional Hospital, Most, Czech Republic; and †††Dermatopathology Unit, Department of Dermatology, Zurich University Hospital, Zurich, Switzerland.
    Extramammary Paget disease (EMPD) is a rare neoplasm usually presenting in the anogenital area, most commonly in the vulva. Adnexal involvement in primary EMPD is a very common feature and serves as a pathway for carcinoma to spread into deeper tissue. The depth of carcinomatous spread along the appendages and the patterns of adnexal involvement were studied in 178 lesions from 146 patients with primary EMPD. Read More

    Genetic Analysis of Mismatch Repair Genes Alterations in Extramammary Paget Disease.
    Am J Surg Pathol 2016 Nov;40(11):1517-1525
    Departments of *Laboratory Medicine †Dermatology **Central Laboratory ††Urology, Huashan Hospital Departments of ‡Pathology ¶Laboratory Medicine ∥Microbiology, Huashan Hospital North §Department of Microbiology, Children's Hospital #Department of Laboratory Medicine, The Fifth People's Hospital of Shanghai, Shanghai Medical School, Fudan University, Shanghai, P.R. China.
    Extramammary Paget disease (EMPD) is a rare cutaneous malignant neoplasm. The familial occurrence of EMPD and the high risk of concomitant secondary tumors in EMPD patients have gained much attention. These findings highlight the importance of genetic alterations in the tumorigenesis of this skin cancer. Read More

    Perianal and Vulvar Extramammary Paget Disease: A Report of Six Cases and Mapping Biopsy of the Anal Canal.
    Ann Dermatol 2016 Oct 30;28(5):624-628. Epub 2016 Sep 30.
    Department of Surgical Oncology, The University of Tokyo Hospital, Tokyo, Japan.
    Treatment of perianal and vulvar extramammary Paget disease (EMPD), rare intraepithelial malignancies, is often challenging because of its potential to spread into the anal canal. However, there is still no consensus regarding the optimal resection margin within the anal canal. Between 2004 and 2014, six patients (three with perianal EMPD and three with vulvar EMPD) in which the spread of Paget cells into the anal canal was highly suspected were referred to our department. Read More

    GATA3 expression in primary vulvar Paget disease: a potential pitfall leading to misdiagnosis of pagetoid urothelial intraepithelial neoplasia.
    Histopathology 2017 Feb 21;70(3):435-441. Epub 2016 Nov 21.
    Department of Pathology, AC Camargo Cancer Center, São Paulo, Brazil.
    Aims: GATA3 has been reported as a specific urothelial marker among organs in the pelvic region, and has been classified as highly sensitive and specific for urothelial and breast carcinomas. Our aim was to verify GATA3 expression in extramammary Paget disease, and to determine whether it can be use to differentiate primary vulvar Paget disease from pagetoid urothelial intraepithelial neoplasia (PUIN). We also analysed HER2 protein expression and HER2 gene amplification and their roles as prognostic factors in extramammary Paget disease. Read More

    Topical imiquimod in the treatment of extramammary Paget's disease: A 10 year retrospective analysis in an Asian tertiary centre.
    Dermatol Ther 2016 Nov 16;29(6):459-462. Epub 2016 Aug 16.
    University Dermatology Clinic, National University Health System, Republic of Singapore.
    Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma usually found in apocrine-rich areas. Although surgery remains standard treatment, topical imiquimod has emerged as a promising drug for the treatment of EMPD in recent years. We present our experience in treating EMPD in Asian skin successfully with topical imiquimod 5% cream, over the past 10 years in our tertiary institution. Read More

    Extramammary Paget disease of the perianal region: the potential role of imiquimod in achieving disease control.
    J Surg Case Rep 2016 Aug 10;2016(8). Epub 2016 Aug 10.
    Department of Colorectal Surgery, Ninewells Hospital and Medical School, Dundee, UK
    Extramammary Paget disease (EMPD) is a rare perineal neoplasia associated with a high rate of local recurrence. Surgical excision is the standard treatment; however, this has high rates of post-operative morbidity in combination with potentially mutilating results. Previous literature has demonstrated good response with imiquimod 5% cream in patients with vulval EMPD, yet its effectiveness in primary perianal disease is unknown. Read More

    RANKL-Expressing Ectopic Extramammary Paget's Disease on the Lower Abdomen.
    Case Rep Dermatol 2016 May-Aug;8(2):130-5. Epub 2016 Jun 6.
    Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
    Ectopic extramammary Paget's disease (EMPD) is a rare variant of EMPD that develops in nonapocrine regions. Since reports about ectopic EMPD are limited, little is known about the biological and immunological background of ectopic EMPD. In this report, we present a case of ectopic EMPD on the lower abdomen that expressed RANKL but lacked the expression of MMP7. Read More

    Perianal Paget's disease: case report and review of the literature.
    Acta Chir Belg 2016 May 26:1-6. Epub 2016 May 26.
    d Department of Pathology , Sint-Jozef Hospital , Bornem , Belgium.
    A peri-anal skin lesion, often eczema-like and with symptoms of pruritus, that does not resolve after classical local therapy should be biopsied. We present a case of peri-anal extramammary Paget's disease (EMDP) and associated anal adenocarcinoma. Reviewing the literature, more than 30% of patients with EMDP present a second primary tumour in their past, present or future history. Read More

    Possible mechanisms of the crosstalk between Langerhans cells and regulatory T cells in extramammary Paget disease by receptor activator of nuclear factor kappa B (RANK) ligand/RANK pathways.
    Br J Dermatol 2016 Jul 14. Epub 2016 Jul 14.
    Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
    Background: Extramammary Paget disease (EMPD) is a skin adenocarcinoma of apocrine gland origin, in which Paget cells express receptor activator of nuclear factor kappa B (RANK) ligand (RANKL) and matrix metalloproteinase (MMP)-7, and release soluble (s)RANKL into the tumour microenvironment. We previously reported that about 60% of the RANK(+) cells among the stromal cells are M2 macrophages, but the identity of the remaining population of RANK(+) cells is still unknown.

    Objectives: To investigate the unknown subpopulation of RANK-expressing cells in EMPD. Read More

    Perineal Paget's disease: A rare disorder and review of literature.
    Ann Med Surg (Lond) 2016 Aug 21;9:50-2. Epub 2016 Jun 21.
    General Surgery Unit, Department of Emergency and Organ Transplantation-University of Bari, Italy.
    Introduction: Extramammary Paget's disease (EMPD) occurs commonly in perineum, vulva and perineal region and is considered as a complex disorder due to different clinical and histological features.

    Presentation Of Case: A 61 years old woman had a dermatologic evaluation for anal itch and underwent a skin biopsy with diagnostic of Paget disease in perianal region. Pelvic magnetic resonance showed a huge tumor which involved skin, derma and gluteus and she firstly refused any surgical treatment. Read More

    Human Epidermal Growth Factor Receptor 2 (HER-2/neu)-Directed Therapy for Rare Metastatic Epithelial Tumors with HER-2 Amplification.
    Case Rep Oncol 2016 May-Aug;9(2):298-304. Epub 2016 Jun 11.
    Division of Hematology-Oncology, Department of Medicine, University of California Los Angeles, Los Angeles, Calif., USA.
    Case 1: A 67-year-old Asian female was diagnosed with locally advanced high-grade salivary duct carcinoma in June 2011. Molecular analysis revealed human epidermal growth factor receptor 2 (HER-2) amplification. She received adjuvant therapy with carboplatin/paclitaxel/ trastuzumab and maintenance of trastuzumab. Read More

    Extramammary paget's disease mimicking localized malignancy on cervical cytology.
    Diagn Cytopathol 2016 Nov 7;44(11):931-934. Epub 2016 Jul 7.
    Department of Gynaecology Oncology, Chris O'Brien Lifehouse, Camperdown, New South Wales, Australia.
    A case of Extramammary Paget's Disease (EMPD) involving the cervix mimicking cervical carcinoma on routine cervical cytology in a 66-year-old woman with a history of recurrent multifocal EMPD involving the vulva, perineum, perianal area, and rectum is reported. The patient had undergone multiple excisions and reconstructions of EMPD, however, had a benign cervical smear history and reported no vaginal bleeding. The conventional papanicolaou smear was reported as concerning for carcinoma with abundant, well preserved material illustrating highly atypical cells with anisonucleosis and dense cytoplasm and focal microacini. Read More

    A proposal for a TNM staging system for extramammary Paget disease: Retrospective analysis of 301 patients with invasive primary tumors.
    J Dermatol Sci 2016 Sep 3;83(3):234-9. Epub 2016 Jun 3.
    University of Tsukuba, Japan.
    Background: Although extramammary Paget disease (EMPD) usually appears as carcinoma in situ, it sometimes becomes invasive (iEMPD) and fatal. However, a TNM staging system for iEMPD has yet to be established.

    Objective: The aim of this study was to establish a TNM staging system for iEMPD. Read More

    Concordance of the HER2 protein and gene status between primary and corresponding lymph node metastatic sites of extramammary Paget disease.
    Clin Exp Metastasis 2016 Oct 11;33(7):687-97. Epub 2016 Jun 11.
    Department of Dermatologic Oncology, National Cancer Center Hospital, Tokyo, Japan.
    Recent studies have reported the overexpression of human epidermal growth factor receptor 2 (HER2) in primary extramammary Paget disease (EMPD). These results indicate that therapies that target HER2 may be useful in treating metastatic EMPD, for which the prognosis is poor. However, there is limited information on the expression and gene amplification of HER2 in metastatic EMPD. Read More

    RANKL expression is a useful marker for differentiation of pagetoid squamous cell carcinoma in situ from extramammary Paget disease.
    J Cutan Pathol 2016 Sep 20;43(9):772-5. Epub 2016 Jun 20.
    Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
    Background: Pagetoid squamous cell carcinoma in situ (SCCIS) is a histopathologic variant of SCCIS composed of cells that display an abundant, pale-staining cytoplasm in a pagetoid distribution within the epidermis. As pagetoid SCCIS is sometimes difficult to differentiate from extramammary Paget disease (EMPD) histopathologically, specific markers for pagetoid SCCIS or EMPD are needed by dermatopathologists.

    Methods: In this report, we employed immunohistochemical staining for receptor of activated nuclear factor kappa ligand (RANKL) and programmed death-ligand 1 (PD-L1) in six cases each of pagetoid SCCIS and EMPD. Read More

    A pilot study of topical imiquimod therapy for the treatment of recurrent extramammary Paget's disease.
    Gynecol Oncol 2016 Jul 5;142(1):139-43. Epub 2016 May 5.
    Gynecology Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA; Weill Cornell Medical College, 1300 York Avenue, New York, NY 10065, USA. Electronic address:
    Objective: The objective of this prospective pilot study was to assess the clinical and histologic effects of topical imiquimod therapy on recurrent extramammary Paget's disease of the vulva.

    Methods: Patients with biopsy-proven recurrent extramammary Paget's disease presenting to the gynecology outpatient services at two participating institutions were recruited for conservative treatment with 5% imiquimod cream from 2007 to 2011. The topical cream was to be applied 3 times per week for 12weeks. Read More

    Penile Analogue of Stratified Mucin-Producing Intraepithelial Lesion of the Cervix: The First Described Case. A Diagnostic Pitfall.
    Am J Dermatopathol 2016 May;38(5):e64-7
    *Department of Pathology, Charles University, Biomedical Center, Faculty of Medicine in Plzen and Charles University Hospital Plzen, Pilsen, Czech Republic; †Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic; ‡Department of Dermatovenereology, Charles University, 2nd Faculty of Medicine and Bulovka Hospital, Prague, Czech Republic; and §Department of Pathology, Charles University, 2nd Faculty of Medicine, Prague, Czech Republic.
    The authors report a case where undifferentiated (classic) penile intraepithelial neoplasia was associated with the presence of goblet cells throughout the full epithelial thickness and which later progressed into an invasive carcinoma. The lesion evolved in three consecutive biopsies from only surface epithelium occupying numerous goblet cells in the first to variably sized solid nodules in the dermis composed of atypical squamous and/or basaloid cells intermixed with numerous goblet cells in the third biopsy. Both cellular components expressed CK7 and p16 protein. Read More

    Multifocal extramammary Paget's disease-associated adenocarcinoma: a rare condition of flexoral skin of multiple sites.
    Dermatol Online J 2016 Jan 15;22(1). Epub 2016 Jan 15.
    University of Texas Health Science Center at Houston.
    Extramammary Paget disease (EMPD) is a rare malignant neoplasm of apocrine sweat glands that is morphologically and histologically identical to Paget disease of the breast. The primary lesion is usually a solitary, well-demarcated, erythematous, scaly plaque that may contain crust, erosions, or ulcerations. The vulva is the most common site, but any area containing apocrine sweat glands may be involved. Read More

    Usefulness of sentinel lymph node biopsy for prognostic prediction in extramammary Paget's disease.
    Eur J Dermatol 2016 Jun;26(3):254-9
    Saitama Medical University, Morohongo 38, Irumagun, 350-0495 Saitama.
    Complete excision of the primary lesion has long been considered the standard treatment for extramammary Paget's disease (EMPD). However, the presence of lymph node metastases has been reported as an important prognostic factor. We evaluated the association between lymph node metastasis and prognosis for EMPD using sentinel lymph node (SLN) biopsy. Read More

    Paget disease of the vulva.
    Crit Rev Oncol Hematol 2016 May 4;101:60-74. Epub 2016 Mar 4.
    Department of Obstetrics & Gynaecology, Radboud university medical center, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands.
    In this review, we provide an overview of the clinical aspects, histopathology, molecular genetics, and treatment options for Vulvar Paget's Disease (VPD), a rare skin disease, most commonly found in postmenopausal Caucasian women. The underlying cause of VPD remains not well understood. VPD is rarely associated with an underlying urogenital, gastrointestinal or vulvar carcinoma. Read More

    A Case of Advanced Extramammary Paget's Disease Successfully Controlled by Monthly but Not Weekly Docetaxel Chemotherapy.
    Case Rep Oncol 2016 Jan-Apr;9(1):6-10. Epub 2016 Jan 8.
    Department of Dermatology, The Jikei University School of Medicine, Tokyo, Japan.
    Extramammary Paget's disease (EMPD) is an uncommon cutaneous adenocarcinoma arising from the apocrine glands within the epidermis or underlying skin appendages in the anogenital and axillary regions. Surgical excision is basically performed as a treatment for EMPD. However, therapeutic options for EMPD in an advanced stage are limited. Read More

    Surgical Treatment of Paget Disease of the Vulva: Prognostic Significance of Stromal Invasion and Surgical Margin Status.
    J Low Genit Tract Dis 2016 Apr;20(2):184-8
    1Gynecologic Oncology Unit, Centro di Riferimento Oncologico, National Cancer Institute, Aviano; 2Woman's Health Sciences Department, Università Politecnica delle Marche, Ancona; and 3Pathology Unit, Centro di Riferimento Oncologico, National Cancer Institute, Aviano, Italy.
    Objective: The aim of the study was to evaluate the risk of recurrence according to the surgical margin status and the presence of invasion or of superficially invasive carcinoma in patients with extramammary Paget disease (EMPD) of the vulva, who underwent elective surgical treatment.

    Materials And Methods: We performed a retrospective analysis of 27 patients with first diagnosis of extramammary Paget disease of the vulva, who underwent primary and elective surgical treatment from January 1989 to December 2014. A p value of less than . Read More

    Syringocystadenocarcinoma Papilliferum In Situ-Like Changes in Extramammary Paget Disease: A Report of 11 Cases.
    Am J Dermatopathol 2016 Dec;38(12):882-886
    *Department of Pathology, Clinical Research and Practical Center for Specialized Oncological Care, Saint Petersburg, Russia; †Department of Pathology, Medical Faculty, Saint Petersburg State University, Russia; ‡Bioptical Laboratory, Pilsen, Czech Republic; §Sikl's Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague, Pilsen, Czech Republic; ¶Department of Histopathology, King Edward Memorial Hospital, Perth, Australia; ‖Department of Pathology, Medical Faculty Hospital, Martin, Slovak Republic; **Pathology and Laboratory Medicine Service, Veterans Administration Medical Center, OK; ††Department of Pathology, University of Oklahoma College of Medicine, Oklahoma City, OK; ‡‡Private Pathology Laboratory, Chomutov, Czech Republic; §§Private Pathology Laboratory, Ostrava, Czech Republic; and ¶¶Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
    The authors report 11 cases of extramammary Paget disease (EMPD), all of which also demonstrated a combination of histological changes highly reminiscent of syringocystadenocarcinoma papilliferum in situ. In addition to the classical features of EMPD, characterized by the intraepidermal spread of individually dispersed neoplastic cells with ample cytoplasm, many of which contained mucin, there were areas of acanthosis with the substitution of spinous layer keratinocytes by neoplastic cells, whereas the native basal cell layer was intact. In addition to acanthosis (and sometimes papillomatosis), the dermal papillae showed a prominent infiltrate of plasma cells, completing the resemblance to syringocystadenocarcinoma papilliferum in situ; this similarity was further enhanced in 2 cases, which showed conspicuous gland formation. Read More

    Syringomatous Structures in Extramammary Paget Disease: A Potential Diagnostic Pitfall.
    Am J Dermatopathol 2016 Sep;38(9):653-7
    *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, BC Cancer Agency, Vancouver, British Columbia, Canada; §Department of Histopathology, King Edward Memorial Hospital, Perth, Australia; ¶Department of Pathology, Division of Dermatopathology, Medical College of Wisconsin, Milwaukee, WI; ‖Sikl's Department of Pathology, Bioptical Laboratory, Pilsen, Czech Republic; **Sikl's Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague, Pilsen, Czech Republic; and ††Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
    Primary extramammary Paget disease (EMPD) is a form of intraepithelial adenocarcinoma. Different morphological changes may accompany EMPD, including the presence of syringoma-like structures. The authors report 10 cases of EMPD, all of which manifested syringoma-like structures within the dermis both in areas involved by the carcinoma and beyond, including at the margins of the excisions. Read More

    Prognostic factors for recurrence and survival among patients with invasive vulvar Paget disease included in the VULCAN study.
    Int J Gynaecol Obstet 2016 Apr 18;133(1):76-9. Epub 2015 Dec 18.
    Gynecologic Oncology Unit, La Paz University Hospital, IdiPAZ, Madrid, Spain. Electronic address:
    Objective: To identify prognostic factors associated with recurrence and overall survival among patients with invasive vulvar Paget disease.

    Methods: An analysis was conducted of patients with invasive vulvar Paget disease included in VULCAN, an international multicenter retrospective study of patients diagnosed with vulvar cancer between January 1, 2001, and December 31, 2005. Data regarding diagnosis, treatment, and follow-up were obtained from the patients' medical records. Read More

    Clinical and pathological characteristics of extramammary Paget's disease: report of 246 Chinese male patients.
    Int J Clin Exp Pathol 2015 1;8(10):13233-40. Epub 2015 Oct 1.
    Department of Laboratory Medicine, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China ; Department of Laboratory Medicine, Huashan Hospital North, Shanghai Medical School, Fudan University Shanghai, P. R. China ; Central Laboratory, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China.
    Extramammary Paget's disease (EMPD) is a rare cutaneous neoplasm. The aim of this study was to elaborate the clinical and pathological features of Chinese EMPD male patients. The study comprised 246 patients with EMPD at our institute from January 1993 to December 2012. Read More

    [Clinicopathological features of extramammary Paget's disease: a report of 75 cases].
    Zhonghua Yi Xue Za Zhi 2015 Jun;95(22):1751-4
    Objective: To evaluate the clinicopathological features of extramammary Paget's disease.

    Methods: Statistical analyses were performed for the clinicopathological features for 75 cases of extramammary Paget's disease from 2004 to 2013.

    Results: Extramammary Paget's disease exhibited a male predominance (5. Read More

    Usefulness of docetaxel as first-line chemotherapy for metastatic extramammary Paget's disease.
    J Dermatol 2016 Jun 25;43(6):633-7. Epub 2015 Nov 25.
    Department of Dermatology, Toranomon Hospital, Tokyo, Japan.
    In invasive extramammary Paget's disease (EMPD), distant metastases may develop and the condition may become fatal; however, no standardized treatment has been established. Although based on only a few cases, several chemotherapy regimens were reported to be promising. We conducted a multicenter, retrospective study to evaluate the efficacy of docetaxel for metastatic EMPD. Read More

    Primary invasive extramammary Paget disease on penoscrotum: a clinicopathological analysis of 41 cases.
    Hum Pathol 2016 Jan 25;47(1):70-7. Epub 2015 Sep 25.
    Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai 200032, People's Republic of China; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, People's Republic of China. Electronic address:
    To investigate the clinicopathological and immunohistochemical features and prognostic factors for invasive extramammary Paget disease (EMPD) on penoscrotum, we described the clinical presentations, histopathology, and follow-up courses of 41 cases. The age of the patients ranged from 42 to 84 years. All the patients were treated with wide surgical excision, and 14 were confirmed to have lymph node metastasis. Read More

    Rare Case of Anal Canal Signet Ring Cell Carcinoma Associated with Perianal and Vulvar Pagetoid Spread.
    J Pathol Transl Med 2016 May 8;50(3):231-7. Epub 2015 Oct 8.
    Department of Pathology, Gachon University Gil Medical Center, Incheon, Korea.
    A 61-year-old woman was referred to surgery for incidentally found colonic polyps during a health examination. Physical examination revealed widespread eczematous skin lesion without pruritus in the perianal and vulvar area. Abdominopelvic computed tomography showed an approximately 4-cm-sized, soft tissue lesion in the right perianal area. Read More

    A case of metastatic adenocarcinoma on a background of penoscrotal extramammary Paget's disease.
    BMJ Case Rep 2015 Sep 30;2015. Epub 2015 Sep 30.
    Department of Urology, University College London Hospital, London, UK.
    A 77-year-old man presented with a chronic lesion located in the left penoscrotal area. Apart from pruritus, bleeding and an occasional discharge from this area, he also reported reduced appetite and weight loss. Examination revealed an ulcerated skin lesion attached to a firm subcutaneous mass. Read More

    Vulval symptoms after the menopause - Not all atrophy!
    Post Reprod Health 2015 Dec 29;21(4):146-50. Epub 2015 Sep 29.
    Frimley Health NHS Trust and St Johns's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, UK
    Vulval and vaginal symptoms are common after the menopause and are frequently assumed to be due to the normal physiological changes that occur at this time. However, there are several important dermatoses that can occur in this patient group which need accurate diagnosis and appropriate management. This review discusses the clinical features and basic management of some of the common vulval problems occurring after the menopause. Read More

    Profunda artery perforator based V-Y rotation advancement flap for total vulvectomy defect reconstruction--A case report and literature review.
    Microsurgery 2015 Nov 24;35(8):668-71. Epub 2015 Sep 24.
    Department of Plastic and Reconstructive Surgery, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan.
    Reconstruction of total vulvectomy defect represents a challenge, and reconstructive methods include skin graft, local skin flap, musculocutanous flap, and pedicled perforator flap. We report a case of a 63-year-old patient affected by extramammary Paget's disease of vulva who underwent total vulvectomy receiving reconstruction with bilateral profunda artery perforator based V-Y rotation advancement flap. The literature about vulva reconstruction was reviewed. Read More

    Androgen receptor, androgen-producing enzymes and their transcription factors in extramammary Paget disease.
    Hum Pathol 2015 Nov 21;46(11):1662-9. Epub 2015 Jul 21.
    Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, 980-8575 Japan.
    Extramammary Paget disease (EMPD) has been known to frequently express androgen receptor (AR). Therefore, androgens could play roles in the biological behavior of Paget cells. 5α-Reductase (5α-red) types 1 and 2 and 17β-hydroxysteroid dehydrogenase type 5 (17β-HSD5) are pivotal in situ regulators of androgen production in androgen-responsive tissues including androgen-dependent neoplasms. Read More

    Psoriasis with extramammary paget disease in a male: a case report.
    Int J Clin Exp Pathol 2015 1;8(7):8642-4. Epub 2015 Jul 1.
    Department of Pathology, 306 Hospital of PLA Beijing, PR China.
    Psoriasis is a chronic inflammatory skin disease that is characterized by erythematous, sharply demarcated papules and plaques covered by scales. Extramammary Paget disease (EMPD) is a uncommon neoplastic condition of apocrine gland-bearing skin and its occurrence in combination with psoriasis is very rare. We report an interesting case of a 61-year-old male with extensive psoriasis presented with penoscrotal EMPD, which was confirmed by histopathological stain. Read More

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