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Indian J Radiol Imaging 2022 Mar 1;32(1):142-144. Epub 2022 May 1.

Department of Gastroenterology and Hepatology, Grande International Hospital, Dhapasi, Kathmandu, Nepal.

When faced with eczematous lesions involving the nipple-areolar complex (NAC), Paget's disease is assumed to be the diagnosis, anything else being considered a "Zebra" necessitating its exclusion on pathology. A middle-aged lady presented with synchronous asymmetrical ulceration of bilateral NAC with pleomorphic calcifications on mammography and simultaneous extensive vascular calcification in bilateral breasts that suggested systemic cause. Calciphylaxis is a rare diagnosis occurring usually due to underlying end-stage renal disease or hyperparathyroidism. Read More

J Natl Compr Canc Netw 2022 Jun;20(6):691-722

28Moffitt Cancer Center.

The therapeutic options for patients with noninvasive or invasive breast cancer are complex and varied. These NCCN Clinical Practice Guidelines for Breast Cancer include recommendations for clinical management of patients with carcinoma in situ, invasive breast cancer, Paget disease, phyllodes tumor, inflammatory breast cancer, and management of breast cancer during pregnancy. The content featured in this issue focuses on the recommendations for overall management of ductal carcinoma in situ and the workup and locoregional management of early stage invasive breast cancer. Read More

Ann Ital Chir 2022 May 5;11. Epub 2022 May 5.

Mammary Paget's disease is a disorder of the nipple-areola complex of the breast that, while rare, is often associated with an underlying carcinoma. The typical aspect is usually an eczematoid change of the nipple or a red and ulcerative nipple's lesion or erythematous and crusted lesion, with or without mass-like lesion and infiltration and inversion of the nipple. It was described at first by Sir James Paget in 1874, [1] who classified the disease in mammary and extramammary type. Read More

Cancer Diagn Progn 2021 Nov-Dec;1(5):387-392. Epub 2021 Nov 3.

Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

Aim: Classically, 'Paget disease' refers to a distinct histological pattern in breast carcinoma. Here, we review the clinicopathological features of anorectal adenocarcinoma with 'pagetoid' spread.

Materials And Methods: Histological and immunohistochemical records for 11 cases of anorectal adenocarcinoma with pagetoid spread among 958 Japanese patients with primary rectal/anal carcinoma were reviewed. Read More

Front Med (Lausanne) 2022 21;9:839954. Epub 2022 Mar 21.

Department of Dermatology, Xiangya Hospital, Central South University, Changsha, China.

Several cases of pigmented mammary Paget's disease (PMPD) mimicking cutaneous malignant melanoma have been reported. In these cases, the tumor cells are colonized by melanocytes, particularly with the presence of a population of melanocytes staining for HMB-45 and S100. Here, we report a case of mammary Paget disease (MPD) which was misdiagnosed as melanoma due to the interpretation of the staining of melanocytic markers S-100, Melan-A, and HMB-45. Read More

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

JAAPA 2022 Apr;35(4):35-38

Raquel Barreto practices in surgical oncology at Memorial Sloan Kettering Cancer Center's ambulatory surgical center in Monmouth, N.J. The author has disclosed no potential conflicts of interest, financial or otherwise.

Abstract: Paget disease of the breast is a rare form of breast cancer that affects the nipple and areolar complex. Clinicians should have a high suspicion for this condition in patients who fail conservative treatment for benign-appearing dermatologic findings regardless of age or sex. This article describes a patient with whose presumed nipple infection was initially thought to be related to her nipple piercing. 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

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

Int J Surg Case Rep 2022 Feb 24;91:106796. Epub 2022 Jan 24.

St John of God Hospital, Midland, 1 Clayton Street, Midland 6056, Western Australia, Australia.

Introduction And Importance: Extra mammary Paget's disease (EMPD) is a rare disease characterised by intraepithelial involvement of the skin by non-squamous carcinoma cells in the vulva, perineum, and scrotum. Secondary EMPD is even rarer and accounts for roughly 25% of cases (Delport, 2012) [1]. Surgery (Mohs/wide local excision) is the standard treatment for primary non-invasive disease, but management of secondary invasive EMPD is less defined as there are no current treatment guidelines. Read More

Int J Surg Pathol 2022 Jan 31:10668969221076550. Epub 2022 Jan 31.

5506Medical College of Wisconsin, 9200 W. Wisconsin Avenue, Milwaukee, WI, USA, 53226-0509.

We report a unique case of a post-menopausal female who presented with a lesion on the areola. A biopsy of the lesion demonstrated a pagetoid intraepithelial neoplasm suggesting a differential diagnosis of mammary Paget disease and pagetoid Bowen disease. Excision of the lesion confirmed the diagnosis of mammary Paget disease with typical histological appearance. 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

Dermatol Reports 2021 Nov 29;13(3):9235. Epub 2021 Jul 29.

Melanoma and Skin Cancer Unit AVNO (Area Vasta Nord Ovest) and Unit of Dermatology, Livorno Hospital, Livorno.

A very rare variant of mammary Paget disease (MPD) is the pigmented MPD, first described in 1956. It is very difficult to distinguish this variant from melanoma both clinically and dermoscopically. The diagnosis is confirmed by histopathology and immunohistochemistry. 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.

Cureus 2021 Nov 2;13(11):e19216. Epub 2021 Nov 2.

Breast Imaging, Baptist Health South Florida, Miami, USA.

Paget's disease of the breast is rare among breast cancers. Secondary Paget's disease of the breast presenting as local recurrence is even rarer, with limited published information on the overall prevalence. In this report, we present a case of secondary Paget's disease of the breast presenting as a pruritic rash and skin changes with normal imaging. 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

Exp Biol Med (Maywood) 2022 02 29;247(3):187-199. Epub 2021 Nov 29.

Experimental Center of Basic Medicine, 26453Wenzhou Medical University, Wenzhou 325000, China.

Paget disease of the breast is an uncommon malignant tumor with an inferior outcome. Therefore, establishing nomograms to predict the survival outcomes of breast Paget disease patients is urgent. Clinicopathological and follow-up data of breast Paget disease patients diagnosed between 2010 and 2016 were retrieved through the Surveillance, Epidemiology, and End Result (SEER) database. Read More

Ann Med Surg (Lond) 2021 Dec 11;72:103035. Epub 2021 Nov 11.

Smart Health Tower, Madam Mittarand Str., Sulaimani, Iraq.

Introduction: Paget's disease of the breast is a rare cutaneous eczema-like condition that occurs in the nipple-areolar complex of the breast. The current study aims to report a rare case of Paget's disease of the breast associated with invasive ductal carcinoma in a male breast.

Case Report: A 54-year-old male presented with ulceration of the left nipple-areolar complex that has been progressing over the last 6 months. Read More

J Natl Compr Canc Netw 2021 05 1;19(5):484-493. Epub 2021 May 1.

26Research Advocacy Network.

The NCCN Guidelines for Breast Cancer include up-to-date guidelines for clinical management of patients with carcinoma in situ, invasive breast cancer, Paget disease, phyllodes tumor, inflammatory breast cancer, male breast cancer, and breast cancer during pregnancy. These guidelines are developed by a multidisciplinary panel of representatives from NCCN Member Institutions with breast cancer-focused expertise in the fields of medical oncology, surgical oncology, radiation oncology, pathology, reconstructive surgery, and patient advocacy. These NCCN Guidelines Insights focus on the most recent updates to recommendations for adjuvant systemic therapy in patients with nonmetastatic, early-stage, hormone receptor-positive, HER2-negative breast cancer. Read More

Cancer Rep (Hoboken) 2021 Oct 27:e1572. Epub 2021 Oct 27.

Department of Pathology, Nepal Cancer Hospital and Research Center, Harisiddhi, Lalitpur, Nepal.

Background: Invasive mammary Paget's disease (MPD) is an extremely rare eczematous eruption on the nipple and areola with an invasion of the dermis by Paget cells. This entity can often be misdiagnosed and overtreated for invasive carcinoma of the breast.

Case: A 34-year woman presented with a 2-year history of right nipple eczema and right axillary lump for a month. Read More

Case Rep Oncol 2021 May-Aug;14(2):1242-1247. Epub 2021 Aug 17.

Department of Breast and Endocrine Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan.

Mammary Paget's disease is a rare malignancy. Mastectomy or breast-conserving surgery has been considered as the standard treatment, while there have been few reports of neoadjuvant chemotherapy (NAC). A 53-year-old woman with erythema and skin ulceration of the left breast was admitted to our institution. Read More

Chin Med J (Engl) 2021 Oct 6;134(20):2483-2485. Epub 2021 Oct 6.

Department of Burn and Plastic Surgery, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, China.

Int J Gynecol Pathol 2021 Sep 27. Epub 2021 Sep 27.

Department of Pathology, Royal Gwent Hospital, Newport (V.I.S.) Department of Histopathology, Portsmouth University Hospitals NHS Trust, Portsmouth (L.B.) Department of Cellular Pathology, University Hospital Wales, Cardiff (G.L.R.) Department of Pathology, Belfast Health and Social Care Trust, Belfast (W.G.M.C.), UK.

Vulval extramammary Paget disease (vEMPD) is an uncommon epithelial malignancy that may arise within the vulva (primary vEMPD) or represent vulval skin involvement by a noncutaneous carcinoma (secondary vEMPD). Primary vEMPD is most often an in situ carcinoma arising within the epidermis but may be associated with dermal invasion (invasive vEMPD) or represent intraepidermal spread of an adenocarcinoma originating in vulval skin adnexa or anogenital mammary-like glands. The latter, termed mammary gland-like adenocarcinoma (MGLA), exhibits morphologic, immunohistochemical, and molecular features of various breast carcinomas but, as far as we are aware, the metaplastic variant of MGLA has not been reported on the vulva. Read More

J Cutan Pathol 2022 Jan 10;49(1):99-102. Epub 2021 Oct 10.

Dermatology Department, Hospital Universitario Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.

Pigmented epidermotropic breast cancer metastases are a rarity, often clinically misdiagnosed as melanocytic lesions. Histopathologically, they show a dermal proliferation of neoplastic metastatic cells that extend to the overlying epidermis in a pattern identical to that seen in primary Paget disease (PD). Differential diagnosis should be established with entities with a similar presentation, such as pigmented mammary PD and malignant melanoma. Read More

Int J Surg Case Rep 2021 Sep 22;86:106234. Epub 2021 Jul 22.

Department of Visceral Surgery, National Teaching Hospital-Hubert Koutoukou Maga, Cotonou, Benin.

Introduction And Importance: Known as a very uncommon disease, erosive adenomatosis of the nipple is a benign neoplasm of the breast. However, its destructive process can have a considerably negative impact on patient's quality of life.

Case Presentation: We report the case of a 45-year-old woman who presented at the visceral surgery department of the National Teaching Hospital of Cotonou-Benin (CNHU-HKM) for a burgeoning mass of the left nipple. Read More

Invest New Drugs 2022 02 31;40(1):194-197. Epub 2021 Aug 31.

Department of Medical Oncology, Kindai University Faculty of Medicine, Osaka-Sayama, Japan.

Extramammary Paget disease (EMPD) is a rare cutaneous adenocarcinoma that usually is of epidermal origin and shows glandular differentiation and that is treated by wide local excision depending on the disease extent. For widely metastatic disease, however, a standard treatment remains to be established. Similar to breast cancer, EMPD has been found to overexpress human epidermal growth factor receptor 2 (HER2) or hormone receptors (HRs). Read More

Diagn Pathol 2021 Aug 1;16(1):69. Epub 2021 Aug 1.

Department of General Surgery, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, No.168 Litang Road, Changping District, Beijing, 102218, China.

Background: Extramammary Paget disease (EMPD) is an uncommon malignancy affecting apocrine gland-bearing skin, such as vulvar, perianal, axillary and penoscrotal areas. Paget cells are sometimes detected outside clinical border in a phenomenon called subclinical extension. Satellite is one of the patterns of subclinical extension which is likely to be invisible. Read More

J Dermatol 2021 Oct 24;48(10):E520-E521. Epub 2021 Jul 24.

Department of Dermato-Oncology/Dermatology, National Hospital Organization Kagoshima Medical Center, Kagoshima, Japan.

Cureus 2021 May 22;13(5):e15161. Epub 2021 May 22.

Hepatobiliary and Pancreatic Surgery, Centro Hospitalar Universitário do Porto, Porto, PRT.

Extramammary Paget disease (EMPD) is a rare form of cancer that involves skin areas rich in apocrine glands. The common sites of occurrence include the vulva, perianal region, perineum, and axilla. Perianal Paget disease (PPD) is a subset of EMPD, which arises in the perianal skin. Read More