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    1673 results match your criteria Paget Disease Extramammary

    1 OF 34

    Lava lake structure and cloud-like structureless area: new clues for diagnosing extramammary Paget disease.
    J Eur Acad Dermatol Venereol 2017 Apr 17. Epub 2017 Apr 17.
    Department of Dermatology, Tokyo Women's Medical University Medical Center East, Tokyo, Japan.
    Extramammary Paget disease (EMPD) is a rare malignant skin neoplasm mostly found in the genital area. Its clinical and histopathological features are similar to mammary Paget disease. The clinical differential diagnosis includes various skin diseases. Read More

    Molecular Subtyping of Mammary-Like Adenocarcinoma of The Vulva Shows Molecular Similarity to Breast Carcinomas.
    Histopathology 2017 Apr 18. Epub 2017 Apr 18.
    Division of Anatomical Pathology, Vancouver General Hospital, Vancouver, Canada.
    Aims: Mammary-like adenocarcinoma (MLA) of the vulva is thought to be derived from vulvar mammary-like glands. We characterized a series of MLA using an immunohistochemical algorithm that identifies the major molecular subtypes of breast cancer.

    Methods And Results: Seven cases of vulval MLA were stained for ER, PR, HER2, Ki-67, EGFR, CK5, nestin and 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 Apr 13. 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 Mar 27. 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 Mar 11. 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

    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

    Enlightening the Pink: Use of Confocal Microscopy in Pink Lesions.
    Dermatol Clin 2016 Oct;34(4):443-458
    Medicine and Medical Specialities Department, Medicine and Health Sciences Faculty, Alcalá University, Univeristy Campus, National road II, 28871- Alcalá de Henares, Madrid, Spain; Dermatology Service, Memorial Sloan-Kettering Cancer Center, 16 E 60th Street, New York, NY 10022, USA.
    Solitary pink lesions can pose a particular challenge to dermatologists because they may be almost or completely featureless clinically and dermoscopically, previously requiring biopsy to exclude malignancy. However, these lesions usually are not particularly challenging histopathologically. Thus, the incorporation of in vivo reflectance confocal microscopy into the clinical practice, which allows for noninvasive examination of the skin at the cellular level revealing features previously seen only on histopathology, is particularly useful for this subset of clinically difficult lesions. Read More

    Intraepithelial Extramammary Paget's Disease of the Vulva: The Clinicopathological Characteristics, Management, and Outcome in a Study of 18 Female Patients.
    Dermatol Surg 2016 Oct;42(10):1142-6
    *Department of Obstetrics and Gynecology, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China; †Department of Obstetrics and Gynecology, Shanghai Medical School, Fudan University, Shanghai, China; ‡Shanghai Key Laboratory of Female Reproductive Endocrine Related Diseases, Shanghai, China; §Department of Pathology, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China.
    Objective: This study aimed to analyze the clinicopathologic characteristics, management, and outcome of patients with intraepithelial extramammary Paget's disease of the vulva (EMPDV).

    Materials And Methods: This retrospective study included medical records and pathological slides of 18 female patients with EMPDV between January 2001 and December 2012. Diagnosis was confirmed using conventional hematoxylin and eosin staining. Read More

    Paget's Disease of the Vulva Treated with Imiquimod: Case Report and Systematic Review of the Literature.
    Gynecol Obstet Invest 2017 22;82(1):1-7. Epub 2016 Sep 22.
    Department of Obstetrics and Gynecology, Ruhr University Bochum, Bochum, Germany.
    Background: Extramammary Paget's disease of the vulva (EPDV) is a rare adenocarcinoma in situ of the vulvar skin and is often resected with involved margins due to its reticular growth pattern. Adjuvant treatment with the immunomodulator imiquimod may be suitable to avoid repeated and mutilating surgery.

    Case Presentation: We present the case of a 73-year-old woman with EPDV, initially treated with surgical resection and re-resection for involved margins. 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

    Extramammary Paget's disease of the scalp presenting with a concurrent basal cell carcinoma.
    Pathology 2016 Oct 26;48(6):623-6. Epub 2016 Aug 26.
    Department of Anatomical Pathology, PathWest, QEII Medical Centre, Perth, WA, Australia; School of Pathology and Laboratory Medicine, University of Western Australia, Perth, WA, Australia. Electronic address:

    Ante-mortem diagnosis of pulmonary tumour thrombotic microangiopathy in a patient with unrecognised extramammary Paget's disease.
    BMJ Case Rep 2016 Aug 26;2016. Epub 2016 Aug 26.
    Department of Internal Medicine, Ippan Zaidan Hojin Kanagawa-ken Keiyukai, Yokohama, Kanagawa, Japan.
    Pulmonary tumour thrombotic microangiopathy (PTTM) resulting in fatal pulmonary hypertension is a rare complication of malignancy. Patients with PTTM generally suffer rapid deterioration of hypoxaemia, and a diagnosis is often only made at autopsy. We report a case of extramammary Paget's disease associated with PTTM. 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 2017 Feb 22;176(2):387-394. Epub 2016 Dec 22.
    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

    Surgical Treatment of Paget Disease of the Vulva: Prognostic Significance of Stromal Invasion and Surgical Margin Status.
    J Low Genit Tract Dis 2016 Jul;20(3):e53-4
    Woman's Health Sciences Department Università Politecnica delle Marche Ancona, Italy Gynecologic Oncology Unit Centro di Riferimento Oncologico National Cancer Institute Aviano, Italy Woman's Health Sciences Department Università Politecnica delle Marche Ancona, Italy

    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

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