Am J Surg Pathol 2017 Jun 13. Epub 2017 Jun 13.

*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

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

Drug Discov Ther 2017 Jun 11. Epub 2017 Jun 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

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

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

JAMA Dermatol 2017 May 10. Epub 2017 May 10.

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

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

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

N Engl J Med 2017 Apr;376(17):e35

Medway NHS Foundation Trust, Gillingham, United Kingdom

J Eur Acad Dermatol Venereol 2017 Apr 17. Epub 2017 Apr 17.

Department of Dermatology, Tokyo Women's Medical University Medical Center East, 116-8567, Tokyo, Japan.

Histopathology 2017 Apr 18. Epub 2017 Apr 18.

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

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

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

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

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

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

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

Br J Dermatol 2017 02;176(2):293-294

Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, NE2 4HH, U.K.

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

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

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

Clin Exp Dermatol 2017 Apr 10;42(3):336-338. Epub 2017 Jan 10.

Department of Dermatology, Fukushima Medical University, 1 Hikariga-oka, Fukushima, 960-1295, Japan.

Br J Dermatol 2016 Dec 27. Epub 2016 Dec 27.

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

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

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

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

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

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

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

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

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

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

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

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

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:

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

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

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

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

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

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

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

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

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

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

J Am Acad Dermatol 2016 Jul;75(1):e9-e10

Department of Dermatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.

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

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

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

J Low Genit Tract Dis 2016 Jul;20(3):e53

University of North Carolina School of Medicine Chapel Hill, North Carolina