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Clin Med Insights Case Rep 2021 10;14:11795476211009251. Epub 2021 Apr 10.

Department of Radiology, Saiseikai Shiga Hospital, Ritto, Shiga, Japan.

Penoscrotal extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma in older male patients, and no effective nonsurgical treatment strategies are currently available. The aim of this study was to report the usefulness of external radiotherapy (RT) for penoscrotal EMPD in an inoperable elderly patient. This report presents the treatment of an 89-year-old man with widespread penoscrotal EMPD. Read More

J Cutan Pathol 2021 Apr 24. Epub 2021 Apr 24.

Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.

Extramammary Paget disease (EMPD) is a rare cutaneous malignancy that typically involves the genital skin and can be primary or associated with an underlying internal malignancy. The typical histopathological appearance of EMPD consists of single or small aggregates of cells with abundant pale cytoplasm and large pleomorphic nuclei, known as Paget cells, scattered throughout the epidermis. We report a case of anogenital EMPD occurring in a 53-year-old man with unusual histopathologic findings of marked epidermal acanthosis, acantholysis, intraepidermal glandular differentiation, and prominent plasma cell-rich fibrovascular cores. Read More

JAAD Case Rep 2021 May 4;11:1-2. Epub 2021 Mar 4.

Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Dermatol Online J 2021 Mar 15;27(3). Epub 2021 Mar 15.

Dermatology Team, Institute of Oncology, Fundación Arturo López Pérez (FALP), Santiago.

Extramammary Paget disease (EMPD) is a rare skin cancer that affects areas with a high concentration of apocrine glands including genital, axillary, and anal skin. When it affects other locations it is called ectopic extramammary Paget disease (E-EMPD) and is uncommon. To date, there are only 45 case reports to the best of our knowledge. Read More

J Ultrasound Med 2021 Apr 15. Epub 2021 Apr 15.

Department of Medical Ultrasound, Shanghai Skin Disease Hospital, Ultrasound Research and Education Institute, Tongji University School of Medicine, Shanghai, China.

Objectives: Pathological invasion level of extramammary Paget disease (EMPD) is strongly related with its risk staging, treatment, and prognosis. However, the current evaluation before treatments fails to evaluate pathological invasion level of EMPD. High-frequency ultrasound (HFUS) may play a key role to solve this problem. Read More

World J Surg Oncol 2021 Apr 12;19(1):110. Epub 2021 Apr 12.

Division of Plastic and Reconstructive Surgery, Department of Surgery, Taipei Veterans General Hospital, 19F, No.201 Shih-Pai RD Sec 2, Taipei, Taiwan.

Background: This study aimed to investigate the survival analysis of extramammary Paget's disease (EMPD) in a Taiwanese population and to provide data for comparison with other studies in various locations and racial populations.

Methods: We retrospectively analyzed the medical records of 63 patients with EMPD who were surgically treated from 2002 to 2019 at a single institution. The primary endpoint was the 5-year overall survival rate of EMPD, and the secondary endpoint was recurrence-free 5-year survival. Read More

Indian J Sex Transm Dis AIDS 2020 Jul-Dec;41(2):210-212. Epub 2020 Nov 11.

Department of OBG, Father Muller Medical College Hospital, Mangalore, Karnataka, India.

Extramammary Paget's disease (EMPD) of the vulva is a rare disease that occurs mainly in postmenopausal women. It can be primary or secondary. Prognosis of primary EMPD is good, but when secondary, we need to find the underlying malignancy as the prognosis depends on it. Read More

Am J Dermatopathol 2021 Mar 30. Epub 2021 Mar 30.

Department of Dermatology, UConn Health, Farmington, CT.

Case Rep Oncol 2021 Jan-Apr;14(1):430-438. Epub 2021 Mar 12.

Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA.

Metastatic primary cutaneous extramammary Paget disease (EMPD) is a rare clinical entity with a 5-year survival <10% and no standard therapy. We report the first case to our knowledge of metastatic EMPD with treatment response to checkpoint inhibitor immunotherapy. The patient had diffusely metastatic disease and previously progressed on cytotoxic chemotherapy and a molecularly targeted agent. Read More

Indian J Dermatol Venereol Leprol 2021 Mar-Apr;87(2):207-213

Department of Dermatologic Surgery, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing.

Background: Due to the clinically poorly delineated unclear margin of extramammary Paget disease, the recurrence rate after surgical resection is high.

Aims: To compare photodynamic diagnosis and photodynamic plus reflectance confocal microscopy diagnosis in determining the tumor margins in patients with extramammary Paget disease.

Methods: Thirty-six patients with histopathologically confirmed primary extramammary Paget disease between January 2017 to June 2018 were included in the study. Read More

Dermatol Pract Concept 2021 Mar 8;11(2):e2021011. Epub 2021 Mar 8.

Department of Gynecology, University of Rome Tor Vergata, Italy.

J Dermatol 2021 May 5;48(5):585-591. Epub 2021 Mar 5.

Department of Dermatology, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.

Although topical therapies are used to treat extramammary Paget's disease (EMPD), reliable treatment outcomes and the effects of these therapies on subsequent surgical treatments are unclear. To assess the clinical outcomes of topical treatment of EMPD and establish the treatment guidelines the medical records of 166 patients diagnosed with EMPD were retrospectively reviewed. The recurrence rate was evaluated according to the previous use of topical agents. Read More

Dermatol Surg 2021 Apr;47(4):473-479

Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.

Background: Extramammary Paget disease (EMPD) poses treatment challenges. Invasive and noninvasive treatment modalities exist with variable success reported. Reflectance confocal microscopy (RCM) is emerging as an adjuvant diagnostic tool. Read More

Intractable Rare Dis Res 2021 Feb;10(1):58-59

Barry University School of Podiatric Medicine, Miami Shores, Florida, USA.

Extramammary Paget's disease (EMPD) is a cancer of the anogenital epithelium. Its origin has been variously attributed to keratinocytes or to Toker cells. Slides of 3 advanced cases of EMPD were incubated with trypsin to retrieve antigens. Read More

Medicine (Baltimore) 2021 Feb;100(5):e24485

Clinical Patology Laboratory, IRCCS Istituto Tumori "Giovanni Paolo II".

Abstract: Paget disease is a complex disorder that can be identified in the breast (mammary Paget disease) or in other locations (extramammary Paget's disease) such as ano-genital skin (Paget disease of the vulva -PVD). This condition is associated with low mortality, but a late diagnosis and recurrence can negatively impact the prognosis. Therefore, the main objective of this study is to evaluate if the human epididymis protein 4 (HE4) and cancer antigen125 (CA125) can promote recognition of PVD in early stages and during the relapses. Read More

Diagn Cytopathol 2021 Feb 15. Epub 2021 Feb 15.

Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Oita, Japan.

Pulmonary tumor thrombotic microangiopathy (PTTM) is a cancer-related pulmonary complication characterized by rapid progression of dyspnea and pulmonary hypertension, occasionally causing sudden death. Given the condition of patients with dyspnea, lung biopsies are limited because of their invasiveness. A 72-year-old man presented with chronic atrial fibrillation and a high right heart load, as determined using ultrasonography. Read More

Clin Nucl Med 2021 04;46(4):342-344

From the Department of Nuclear Medicine, Fudan University Shanghai Cancer Center; Center for Biomedical Imaging, Fudan University; and Shanghai Engineering Research Center of Molecular Imaging Probes, Shanghai, China.

Abstract: Extramammary Paget disease is a rare skin intraepithelial adenocarcinoma. Our case presents 68Ga-fibroblast activation protein inhibitor and 18F-FDG PET/CT imaging in a patient with perineum extramammary Paget disease. Compared with 18F-FDG PET/CT, the primary tumor, enlarged pelvic lymph nodes and right maxillofacial lesion showed higher uptake in 68Ga-fibroblast activation protein inhibitor PET/CT. Read More

BMJ Case Rep 2021 Feb 4;14(2). Epub 2021 Feb 4.

Surgery, Tras-os-montes and Alto Douro Hospital Centre, Vila Real, Portugal.

Perianal Paget disease (PPD) is a rare neoplastic condition defined by the presence of atypical Paget cells in the perianal skin, the aetiology of which remains largely unknown. It can be divided in primary forms, arising as an intraepithelial disease or manifestation of an underlying skin adenocarcinoma or secondary forms resulting from epidermotropic spread or metastasis of a concealed carcinoma. Indeed, because of its rarity, clear options regarding the treatment of these patients are yet to be clarified. Read More

Obstet Gynecol Surv 2021 Jan;76(1):55-62

Professor, Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, The University of North Carolina at Chapel Hill, Chapel Hill, NC.

Importance: Vulvar intraepithelial neoplasia (VIN) represents an increasingly common, yet challenging diagnosis that shares many common risk factors with cervical intraepithelial neoplasia. However, unlike cervical intraepithelial neoplasia, effective screening and treatment strategies are much less defined for patients with VIN.

Objective: The objective of this article is to review the underlying risk factors leading to the development of VIN, identify special populations at risk for VIN, and outline acceptable treatment strategies. Read More

J Am Acad Dermatol 2021 Jan 19. Epub 2021 Jan 19.

Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.

Dermatol Surg 2021 May;47(5):613-617

Department of Dermatology, Gangnam Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.

Background: Several studies have reported the presence of hypopigmentation in extramammary Paget disease (EMPD). However, an in-depth analysis regarding its clinical implication is lacking.

Objective: To evaluate the clinical characteristics of EMPD in the Korean population and to determine the implication of hypopigmentation on clinical outcomes. Read More

Ann Dermatol Venereol 2021 Jun 16;148(2):71-76. Epub 2021 Jan 16.

Department of Dermatology, Hôpital Saint-Louis, Paris, France.

Paget's disease (PD) denotes an initially intra-epidermal adenocarcinoma that can later invade the dermis and metastasise. Among the extramammary forms of PD (EMPD), penoscrotal presentations are rarer than the vulvar and perianal forms. Once diagnosis has been confirmed by histopathological examination, a search for associated neoplasia must be conducted, although penoscrotal EMPD is less frequently associated with underlying neoplasia than mammary PD (MPD). Read More

Ann Pathol 2021 Jan 9. Epub 2021 Jan 9.

Cabinet de dermatopathologie Mathurin Moreau, 75019 Paris, France.

Extramammary Paget's disease is a rare malignancy. The underlying cause of VPD remains not well understood. We provide an overview of the histopathology of vulvar Paget's disease, with or without invasion. Read More

Anticancer Res 2021 Jan;41(1):219-226

Department of Pathology, Kurume University School of Medicine, Kurume, Japan.

Background: Extramammary Paget's disease (EMPD) is a type of carcinoma that usually progresses slowly but may cause metastasis and subsequent death of patients. We investigated the relationship between the expression of programmed death-ligand 1 (PD-L1)/programmed death-ligand 2 (PD-L2) and stromal CD8 tumor-infiltrating lymphocytes (TILs) in EMPD and clinicopathological findings, including prognosis.

Materials And Methods: We examined 47 cases of EMPD and performed immunohistochemical staining of formalin-fixed paraffin-embedded full-face sections. Read More

J Breast Cancer 2020 Dec 3;23(6):665-671. Epub 2020 Jun 3.

Division of Breast Surgery, Department of Surgery, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.

Bilateral axillary Paget's disease in men is a rare occurrence with limited reports on its diagnosis, treatment, and prognosis. Here, we report the case of a 55-year-old Korean male, who presented with a palpable mass and eczematous skin lesion on the left axilla. An incisional biopsy and histopathologic examination indicated invasive ductal carcinoma with Paget's disease arising in the accessory breast. Read More

Dermatol Reports 2020 Sep 22;12(2):8841. Epub 2020 Oct 22.

Medical Oncology Department, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental.

Extramammary Paget Disease (EMPD) is an often-misdiagnosed rare disorder, whose cause remains unknown. Diagnosis is confirmed by skin biopsy. Primary treatment for EMPD is surgery. Read More

Am J Dermatopathol 2020 Dec 29;Publish Ahead of Print. Epub 2020 Dec 29.

Department of Dermatology, and Diagnostic Pathology, Shizuoka Cancer Center Hospital, Sunto, Japan; Department of Pathology, Tokyo Metropolitan Cancer and Infectious Disease Center Komagome Hospital, Tokyo, Japan; Department of Pathology, Itabashi Central Clinical Laboratory, Tokyo, Japan; Department of Diagnostic Pathology and Cytology, Osaka International Cancer Institute, Osaka, Japan; and Department of Dermatology, Hyogo Cancer Center, Akashi, Japan.

Abstract: This study aimed to identify the clinical and histopathological characteristics of secondary extramammary Paget disease (EMPD) with underlying anorectal adenocarcinoma so as to differentiate it from primary cutaneous EMPD. Seventeen and 8 cases of primary and secondary EMPD with anorectal adenocarcinoma, respectively, were retrieved from the pathology archive and the clinical and histopathological features reviewed. The tumor samples from 21 cases were totally resected specimens, whereas 3 and 1 of secondary and primary cases were punch biopsied, respectively. Read More

Int J Mol Sci 2020 Dec 27;22(1). Epub 2020 Dec 27.

Division of Gynecology and Obstetrics 1, Department of Surgical Sciences, "City of Health and Science University Hospital", University of Turin, 10126 Turin, Italy.

Vulvar cancer (VC) is a rare neoplasm, usually arising in postmenopausal women, although human papilloma virus (HPV)-associated VC usually develop in younger women. Incidences of VCs are rising in many countries. Surgery is the cornerstone of early-stage VC management, whereas therapies for advanced VC are multimodal and not standardized, combining chemotherapy and radiotherapy to avoid exenterative surgery. Read More

Medicine (Baltimore) 2020 Dec;99(51):e23443

Department of Breast Surgery, The First Affiliated Hospital of China Medical University.

Backgound: This study aims to identify the expression of lipoma preferred partner (LPP) in Paget disease (PD) and to further understand the pathogenesis of PD.

Methods: Tissue microarray was used to evaluate the expression of LPP by immunohistochemistry in 40 PD patients. The results of LPP expression were combined with clinical and histopathological characteristics. Read More