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Urology 2022 May 10. Epub 2022 May 10.

Department of Dermatology, Mayo Clinic; Rochester, MN. Electronic address:

Objectives: To describe local recurrence rates and patient reported outcomes when Mohs micrographic surgery with cytokeratin-7 immunostains (MMS-CK7) is included in the interdisciplinary management of extramammary Paget's Disease (EMPD).

Methods: A retrospective study was conducted of EMPD treated with MMS-CK7 as part of an interdisciplinary team at an academic medical center between 2009 and 2016. Local recurrence rates and patient-reported outcomes were determined by record review and patient surveys. Read More

Aust J Gen Pract 2022 May;51(5):351-352

MBBS (Hons), MMed (ClinEpi), FACD, Consultant Dermatologist, Department of Dermatology, Liverpool Hospital, Liverpool, NSW; Ingham Institute of Applied Medical Sciences, Liverpool, NSW; University of NSW, Sydney, NSW.

JAAD Case Rep 2022 May 22;23:87-89. Epub 2022 Mar 22.

Departments of Dermatology, Mayo Clinic, Jacksonville, Florida.

Expert Opin Biol Ther 2022 05 16;22(5):673-674. Epub 2022 Apr 16.

Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

World J Surg Oncol 2022 Apr 4;20(1):109. Epub 2022 Apr 4.

Chongqing Key Laboratory of Translational Research for Cancer Metastasis and Individualized Treatment, Chongqing University Cancer Hospital, Chongqing, 400030, China.

Background: Male cases of accessory breast carcinoma and sweat gland carcinoma associated with extramammary Paget's disease of the axilla are uncommon. In clinical diagnosis and treatment, it is necessary to determine the disease carefully and make a reasonable treatment strategy according to the patient's situation.

Case Presentation: We described two male cases of the special tumor with an axillary mass as the first clinical symptom, one of which was diagnosed as accessory breast cancer and the other as sweat gland cancer associated with extramammary Paget's disease. Read More

Curr Treat Options Oncol 2022 Jun 4;23(6):818-830. Epub 2022 Apr 4.

Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.

Opinion Statement: Extramammary Paget's disease (EMPD) is a rare neoplastic disease affecting areas rich in apocrine glands in the elderly. EMPD clinically resembles a benign inflammatory skin disease, and ill-defined tumor borders can lead to misdiagnosis and incomplete excision. Several prognostic factors have been reported, including nodule formation, tumor thickness, tumor invasion, lymphovascular invasion, and a perianal location, which are characteristic of primary tumors. Read More

Photodiagnosis Photodyn Ther 2022 Mar 31:102841. Epub 2022 Mar 31.

Department of Plastic & Cosmetic Surgery, Daping Hospital, Army Medical University, Chongqing 400042, PR China. Electronic address:

Background: In recent years, photodynamic diagnosis (PDD) has been a technique that plays a pivotal role in visualizing tumor size during the assessment of surgery. Photodynamic therapy (PDT) is a developing treatment method apart from surgery, chemotherapy, radiotherapy, and immunotherapy and is approved for several types of tumors and nonmalignant diseases. The purpose of this study was to illustrate the efficiency and safety of PDD-guided tumor excision combined with ALA-PDT in patients with extramammary Paget's disease (EMPD). Read More

BMJ Case Rep 2022 Mar 29;15(3). Epub 2022 Mar 29.

Plastic and Reconstructive Surgery, Royal Free Hospital, London, UK.

Ectopic extramammary Paget's disease describes an exceedingly rare intraepithelial adenocarcinoma arising within non-apocrine tissues. We present a case report of E-EPMD arising on the lower abdomen without underlying secondary malignancy in a 56-year-old female patient. We performed a wide local excision of the lesion with subsequent mini abdominoplasty reconstruction. Read More

Cir Esp (Engl Ed) 2022 03;100(3):175-177

General and Digestive Surgery Department, Colorectal Surgery Unit, Donostia University Hospital, Biodonostia Research Institute, Beguiristain Doktorea Pasealekua s/n, 20014 Donostia-San Sebastián, Spain.

Front Med (Lausanne) 2022 25;9:839786. Epub 2022 Feb 25.

Center for Microscopy-Microanalysis and Information Processing, University Politehnica of Bucharest, Bucharest, Romania.

Two-photon microscopy techniques are non-linear optical imaging methods which are gaining momentum in the investigation of fixed tissue sections, fresh tissue or even for experiments. Two-photon excited fluorescence and second harmonic generation are two non-linear optical contrast mechanisms which can be simultaneously used for offering complementary information on the tissue architecture. While the former can originate from endogenous autofluorescence sources (e. Read More

J Cutan Pathol 2022 Mar 11. Epub 2022 Mar 11.

Laboratory Medicine Program, University Health Network and Princess Margaret Cancer Center, Toronto, Ontario, Canada.

Background: Extramammary Paget disease (EMPD) is an uncommon disease affecting older men and women. Clinically, it appears as a plaque lesion with an erythematous or leukoplakic background in regions with abundant apocrine glands such as female external genitalia, perineum, scrotum, and penis.

Methods: We are presenting an 85-year-old patient with recurrent erythematous plaque lesions involving the penis and known to have urothelial carcinoma (UC) in situ of the bladder. Read More

Rom J Morphol Embryol 2021 Jul-Sep;62(3):757-763

Department of Pathophysiology, George Emil Palade University of Medicine, Pharmacy, Science and Technology of Târgu Mureş, Romania;

Introduction: Neoplasms with pagetoid features are a category of rare lesions defined by the presence of atypical cells at different levels of the epidermis. The most important diseases within this category are mammary Paget disease (MPD), extramammary Paget disease (EMPD), Bowen's disease, in situ melanoma, and pagetoid reticulosis.

Aim: The aim of this analysis was to describe the importance of the cytokeratin 8∕18 (CK 8∕18) immunostaining in diagnosing MPD and EMPD and differentiating them from other lesions. Read More

J Dermatol 2022 May 6;49(5):508-514. Epub 2022 Mar 6.

Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.

Although the prognosis of patients with extramammary Paget's disease (EMPD) treated with radical resection is good, the prognosis of EMPD with distant metastasis is very poor. PIK3CA mutations predict a good response to PIK3CA inhibitors. The aim of this study was to investigate the occurrence rate of PIK3CA mutations (including multiple mutations [MM]) related to the intertumor and intratumor heterogeneity in EMPD and to evaluate the correlation between these mutations and clinical parameters of EMPD. Read More

Br J Hosp Med (Lond) 2022 Feb 10;83(2):1-12. Epub 2022 Feb 10.

Department of Gynaecology Oncology, The Royal Wolverhampton NHS Trust, Wolverhampton, UK.

Extra-mammary Paget's disease is a rare cancer affecting the anogenital region and can present with a myriad of symptoms. In women, the diagnosis of Paget's disease can be challenging as it mimics several other vulval conditions. It is important to promptly recognise this disease because of its potential association with synchronous tumours, such as colorectal adenocarcinoma. Read More

Ann Med Surg (Lond) 2022 Feb 28;74:103320. Epub 2022 Jan 28.

Department of Orthopedics and Reconstruction Surgery, National Academy of Medical Sciences, Kathmandu, Nepal.

Introduction: and Importance: Extra Mammary Paget's disease (EMPD) of the vulva, a rare postmenopausal entity, is responsible for less than 1% of all vulvar neoplasms. Invasive EMPD of the vulva with underlying squamous cell carcinoma is even rare.

Case Presentation: A 70-year-old para 5 postmenopausal lady presented with a history of vulvar itching and a gradually progressive reddish lesion on genitals unresolved by topical therapies for one year. Read More

Eur J Dermatol 2022 Feb;32(1):107-112

Department of Dermatology and Venerology, Peking University First Hospital, Beijing, China, Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China, National Clinical Research Center for Skin and Immune Diseases, Beijing, China.

Extramammary Paget's disease (EMPD) frequently extends beyond clinical borders, causing a high recurrence rate. Mohs micrographic surgery (MMS) has been used for management of EMPD, but its efficiency is compromised by technical limitations inherent in MMS. To identify clinicopathologic parameters of predictive value regarding MMS final margin width (FMW) for EMPD, and provide some preliminary guidance in selecting initial surgical margin width for improved efficiency. Read More

Clin Case Rep 2022 Feb 7;10(2):e05356. Epub 2022 Feb 7.

Skin Research Center Shahid Beheshti University of Medical Sciences Tehran Iran.

A 34-year-old female patient presented with recurrent bilateral hypopigmented macules on the labia majora. The lesions were treated with topical steroids, which led to mild improvement, but erosive plaques developed after discontinuing the treatment. Histopathological findings were compatible with extramammary Paget disease (EMPD), which was treated with radical vulvectomy with no recurrence in the next months of follow-up. Read More

Arch Gynecol Obstet 2022 Jan 27. Epub 2022 Jan 27.

Unità di Gineco-Patologia e Patologia Mammaria, Dipartimento Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Introduction: Paget disease is a rare neoplasm of the skin that mainly involves the vulvar region. Vulvar Paget's disease (VPD) can spread beyond the apparent edges of the lesion resulting in a high risk of involved surgical margins. Our aim is to verify the efficacy of a preoperative vulvo-vaginal intensive clock mapping in the prediction of the invasiveness and the extension of VPD. Read More

JAMA Oncol 2022 Apr;8(4):618-628

Gynecology Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York.

Importance: Extramammary Paget disease (EMPD) is a frequently recurring malignant neoplasm with metastatic potential that presents in older adults on the genital, perianal, and axillary skin. Extramammary Paget disease can precede or occur along with internal malignant neoplasms.

Objective: To develop recommendations for the care of adults with EMPD. Read More

Australas J Dermatol 2022 Feb 29;63(1):115-118. Epub 2021 Dec 29.

Department of Dermatology, College of Medicine, Hallym University, Anyang, Korea.

Int J Surg Case Rep 2022 Jan 20;90:106706. Epub 2021 Dec 20.

Department of Obstetrics and Gynecology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan.

Introduction: Patients who have experienced vertical rectus abdominis muscle (VRAM) flap reconstruction are under risk of the abdominal incisional hernia (donor site hernia), and laparotomy increases the risk.

Presentation Of The Case: We present the case of a 71-year-old nulliparous woman who experienced radical vulvectomy against extramammary Paget disease and reconstructive surgery with VRAM flap 7 years ago and developed right ovarian tumor. In this case, right ovarian tumor was removed with laparoscopic surgery, instead of laparotomy, and the risk of incisional hernia was successfully minimized. Read More

Case Rep Oncol 2021 Sep-Dec;14(3):1591-1595. Epub 2021 Nov 8.

Department of Dermatology, Mie University, Graduate School of Medicine, Tsu, Japan.

We present a case of extramammary Paget's disease with bilateral inguinal lymph node metastasis treated by monthly docetaxel chemotherapy. He has also well-controlled psoriasis vulgaris for 20 years. One day after completing monthly chemotherapy, cellulitis by Group G occurred on both legs, resulting in septic shock and disseminated intravascular coagulation. Read More

JAAD Case Rep 2022 Jan 17;19:51-53. Epub 2021 Nov 17.

Department of Gynaecological Oncology, KK Women's and Children's Hospital, Singapore, Singapore.

Acta Derm Venereol 2022 Jan 26;102:adv00636. Epub 2022 Jan 26.

Department of Dermatology, Faculty of Medicine, The University of Tokyo, , Japan.

Extramammary Paget's disease (EMPD) is a rare cutaneous adenocarcinoma with unfavourable prognosis once it becomes invasive. A tumour marker that reflects disease progression is required for adequate management of EMPD. Cytokeratin 18 is highly expressed in many types of cancer and its soluble forms are detected by M30 (for caspase-cleaved form) and M65 (for both caspase-cleaved and intact forms) assays. Read More

BMC Med Genomics 2021 12 11;14(1):291. Epub 2021 Dec 11.

Department of Urology, Shanghai 9Th People's Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, 200011, China.

Background: Extramammary Paget's disease (EMPD) is a rare malignant intraepidermal adenocarcinoma that is poorly understood. Regulatory long noncoding RNAs (lncRNAs) are characterized in many species and shown to be involved in processes such as development and pathologies, revealing a new layer of regulation in different diseases, especially in cancer studies. In the present study, we used high-throughput sequencing to reveal the lncRNA-mRNA interaction network in extramammary Paget's disease. Read More

Gynecol Oncol 2022 02 7;164(2):386-392. Epub 2021 Dec 7.

The Korea Central Cancer Registry, National Cancer Center, Goyang, Republic of Korea; Division of Cancer Registration and Surveillance, National Cancer Control Institute, National Cancer Center, Goyang, Republic of Korea. Electronic address:

Objective: To report 20-year trends in incidence and survival of vulvar cancer in Korea.

Methods: Using data from the Korean Central Cancer Registry, age-standardized incidence rates (ASRs) and annual percentage changes (APCs) were calculated. Net survival (NS) was estimated by the Pohar-Perme method, and conditional net survival (CNS) was calculated. Read More

Photodiagnosis Photodyn Ther 2022 Mar 7;37:102670. Epub 2021 Dec 7.

Institute of Photomedicine, Shanghai Skin Disease Hospital, Tongji University School of Medicine, Shanghai, China. Electronic address:

Background: Extramammary Paget's Disease (EMPD) is an intraepithelial cancer that is prone to recurrence and sometimes refractory to therapy. A few EMPD cases have been treated with Photodynamic therapy (PDT), which reported high complete remission (CR) rates and low recurrence with hematoporphyrin derivatives (HpD) The aim of this study was to further explore the efficacy and safety of HpD-PDT for EMPD patients.

Methods: Open-label, single arm, pilot study was designed to investigate the role of HpD-PDT in EMPD. Read More

Cancer Sci 2022 Feb 13;113(2):802-807. Epub 2021 Dec 13.

Department of Dermatology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.

Extramammary Paget's disease (EMPD) is a rare adnexal neoplasm commonly seen in the genital areas among the senior population. The prognosis of advanced EMPD is not favorable; thus, the development of potential treatments has long been sought. Cyclin-dependent kinase (CDK) 4/6 inhibitors such as abemaciclib and palbociclib have been proven effective against metastatic breast cancer; however, no studies have addressed CDK4/6 inhibitors as an EMPD treatment. Read More

World J Surg Oncol 2021 Dec 2;19(1):336. Epub 2021 Dec 2.

Ningbo Pathological Diagnosis Center, Ningbo, 315000, China.

Background: Perianal Paget's disease (PPD) is a rare malignancy, often associated with an underlying adenocarcinoma and a poor prognosis.

Case Presentation: A 69-year-old female was presented with a history of perianal pruritus for 6 months and enlarged inguinal lymph nodes in the left side. Paget cells were confirmed by pathology after a wide excision of perianal skin. Read More

Dermatol Surg 2021 12;47(12):1654-1655

Sydney Melanoma Diagnostic Centre, Camperdown, New South Wales, Australia.