Paget's disease of the breast: Presentation, treatment, and outcomes in a modern cohort.

BACKGROUND: Surgical management for Paget's disease (PD) of the breast is controversial. This study aims to assess outcomes of PD patients based on procedure type and determine the reliability of imaging in estimating disease extent. METHODS: A retrospective review analyzed clinicopathologic data of PD patients between 2009 and 2022. Pre-operative imaging size (PIS) was compared to post-operative pathology size (PPS) looking at concordance. RESULTS: Thirty patients had PD, 21 underwent total mastectomy (TM) and 9 breast conserving surgery (BCS). Seventeen patients (56.7 â€‹%) had a final diagnosis of invasive cancer (14 â€‹TM, 3 BCS), with no local recurrences. Only 6/19 (31.6 â€‹%) patients with positive findings on ultrasound/mammogram had concordance between PIS and PPS. There were no breast/chest wall recurrences with a median follow up of 35.9 months. CONCLUSION: Ultrasound and mammogram had poor concordance with pathological size. BCS is feasible in select patients. MRI may help guide management.

Male Mammary Paget Disease: A Tale of 2 Contrasting Cases.

Mammary Paget disease (MPD) comprises 1.45% all male breast cancers, compared with only 0.68% of all female breast cancers. Patients usually present in the fifth and sixth decades of life with ulceration, eczematous changes, discharge, bleeding, itching, and induration of the nipple and areola. Typically, there is a delay in definitive diagnosis and treatment from the onset of symptoms because most patients are initially treated for a rash. At the time of diagnosis, about half of the patients may have palpable breast mass, positive lymph nodes, or both. In this article, we present 2 cases of male MPD representing the extremes of clinical, radiologic, and histopathologic spectrum of the disease. One patient presented with a rash of the nipple of several months duration without an underlying lesion, whereas the other presented with sensitivity and pain of the nipple for 1 year and an underlying mass. Biopsies were diagnostic of MPD in both cases, and definitive surgery revealed an underlying ductal carcinoma in situ in the first case and an invasive ductal carcinoma in the second, highlighting the importance of early biopsy to initiate appropriate management.

Mammary Paget's disease diagnosed with reflectance confocal microscopy.

Mammary Paget's disease (MPD) is a rare breast carcinoma represented with an eczematoid cutaneous manifestation. Imitating inflammatory or infectious diseases, it makes early diagnosis and prompt treatment difficult. Non-invasive imaging examinations such as reflectance confocal microscopy (RCM) could extend assistance in making a diagnosis because of its near-cellular resolution in skin diseases. Herein, the RCM feature of two cases of MPD, and the corresponding dermoscopy, ultrasonography examination and immunohistochemistry staining results were described.

Advanced lesions of synchronous bilateral mammary Paget's disease: a case report.

BACKGROUND: Mammary Paget's disease is an eczematous eruption on the nipple and areola with underlying breast malignancy. It is often misinterpreted as chronic dermatitis or psoriasis causing a delayed diagnosis. Synchronous bilateral mammary Paget's disease is exceptionally rare and an advanced case with underlying invasive carcinoma might require long-term treatment and follow-up that could affect a patient's physical, psychological, and social aspects of well-being. CASE PRESENTATION: A 54-year-old Javanese woman presented in our clinic with a 2-year history of itching and chronic eczema in both areolae. Bilateral nipple retraction and retro-areolar palpable lumps were observed during the first presentation. Breast ultrasound revealed hypoechoic lesions in her left and right breasts. Mammograms showed an irregular hyperdense lesion and multiple microcalcifications. Histopathology from biopsy and bilateral mastectomy demonstrated infiltration of large Paget's cells in the epidermis of the areola with underlying lesions of invasive ductal carcinoma, diagnosed solid type with high nuclear grade and negative expression of estrogen receptor and progesterone receptor, with positive expression of human epidermal growth receptor-2(HER2) and Ki-67 (45%). CONCLUSIONS: In a patient with suspicious chronic inflammation of the nipple and areolae, prompt biopsy should be performed to avoid a delayed diagnosis of any malignant breast lesion.

Nipple Ultrasound: A Pictorial Essay.

Ultrasound (US) is an attractive diagnostic approach to identify both common and uncommon nipple pathologies, such as duct ectasia, nipple abscess, nipple leiomyoma, nipple adenoma, fibroepithelial polyp, ductal carcinoma in situ (restricted to nipple), invasive carcinoma, and Paget's disease. US is the reliable first-line imaging technique to assess nipple pathologies. It is useful to identify and characterize nipple lesions. Additionally, we have presented the mammography and MRI outcomes correlated with histopathologic features for the relevant cases.

Dermoscopic features of mammary Paget's disease: a retrospective case-control study by the International Dermoscopy Society.

BACKGROUND: Mammary Paget's disease (MPD) is a rare intraepidermal adenocarcinoma of the nipple-areola complex, associated with an underlying breast cancer in approximately 90% of cases. Delayed diagnosis of MPD is common. Its dermoscopic features have been ill defined in the literature. OBJECTIVES: To determine the clinical and dermoscopic features of MPD versus other dermatologic entities that involve nipple and areola. METHODS: Members of the IDS were invited to submit any case of histologically confirmed MPD, as well as other benign and malignant dermatoses that involve the nipple and areola complex. A standardized evaluation of the dermoscopic images was performed and the results were statistically analyzed. RESULTS: Sixty-five lesions were included in the study, 22 (33.8%) of them MPD and 43 (66.2%) controls. The most frequent dermoscopic criteria of MPD were white scales (86.4%) and pink structureless areas (81.8%), followed by dotted vessels (72.7%), erosion/ulceration (68.2%) and white shiny lines (63.6%). The multivariate analysis showed that white scales and pink structureless areas were significant predictors of MPD, posing a 68-fold and a 31-fold probability of MPD, respectively. Split of the population into pigmented and non-pigmented lesions showed that in pigmented MPD, pink structureless areas, white lines and grey granules and dots are positive predictors of the disease. Among non-pigmented lesions, pink structureless areas, white lines, erosion/ulceration and white scales served as predictors of MPD. CONCLUSIONS: The most frequent profile of an individual with MPD is an elderly female with unilateral, asymptomatic, erythematous plaque of the nipple, dermoscopically displaying pink structureless areas, fine white scales, dotted and a few short linear vessels. In case of pigmentation we may also observe brown structureless areas and pigmented granules. LIMITATIONS: Small sample size, retrospective design.

Nipple and areola lesions: review of dermoscopy and reflectance confocal microscopy features.

The differential diagnosis of nipple and areola complex (NAC) lesions encompasses a large spectrum of conditions from benign tumours to inflammatory diseases that could be challenging to recognize on clinical ground. While melanoma (MM) of the NAC is exceedingly rare, benign lesions are more frequent but could be difficult to distinguish from MM. Besides MM, other malignant tumours can affect this area and in particular Paget's disease (PD). For clinically doubtful lesions, biopsy is required, with possible functional and aesthetic consequences in this sensitive area. Dermoscopy and reflectance confocal microscopy (RCM) are widely used techniques for the diagnosis of many skin lesions, but their use for NAC lesions is not well established. The objective of this study was to evaluate current literature on these imaging techniques for NAC lesions. We searched in Medline, PubMed and Cochrane database all studies up to November 2018 dealing with dermoscopy, RCM and this special site. We found that the most described malignant tumour was PD and that only two primary MMs of the NAC have been reported with these imaging techniques. Although there are few data on diagnostic accuracy of non-invasive imaging techniques for NAC lesions, it seems that dermoscopy and RCM can add relevant information to be integrated with clinical examination for the diagnosis of NAC lesions and in particular for the differential diagnosis of PD and eczema.

Sonographic visualization of nipple blood flow can help differentiate Paget disease from benign eczematous nipple lesions.

PURPOSE: Paget disease of the breast is a rare cancer that originates from the nipple-areolar complex. It is often overlooked and misdiagnosed as benign chronic eczema of the nipple. We aimed to retrospectively verify whether blood flow analysis using Doppler sonography was useful for detecting the presence of Paget disease. METHODS: In this retrospective study, 12 patients with pathologically proven unilateral nipple eczematous lesions (seven with Paget disease and five with simple dermatitis) were included. Nipple blood flow signal was observed using Doppler sonography, and the detected blood flow signals were quantified using digitally recorded images. Quantified blood flow ratio and pathologically examined capillary density were evaluated between affected and unaffected nipples. Findings of mammography, grayscale sonography, and contrast-enhanced magnetic resonance imaging (CE-MRI) were reviewed. RESULTS: In patients with Paget disease, Doppler effects in the affected nipple were more clearly visible than those in the unaffected nipple. These effects were sufficiently visible to identify Paget disease. No obvious effects were observed in the affected and unaffected nipples of simple dermatitis. The quantified blood flow ratio and pathologically examined capillary density were significantly higher for the Paget lesion than those for the non-Paget lesion. The sensitivity of CE-MRI and Doppler sonography was markedly correlated, revealing blood flow changes in the nipple lesions of Paget disease. CONCLUSION: Doppler sonography visualized the proliferation of blood vessels in Paget lesions. The visualization of increased nipple blood flow using Doppler sonography is a simple and low-cost method that provides useful data for identifying Paget disease during routine medical care.

Invasive Solid Papillary Carcinoma of the Nipple With Pagetoid Extension and Nodal Metastasis.

We report a case of invasive solid papillary carcinoma (SPC) of the nipple with Pagetoid extension to the skin and lymph node metastasis. SPC is an uncommon primary breast cancer accounting for less than 1% of all breast cancers. Only 2 cases occurring in the nipple have been reported. However, both cases were without Pagetoid extension or lymph node metastasis. The presently reported tumor consisted of irregularly shaped solid cell nests with delicate fibrovascular cores. The tumor cells had round nuclei with low-grade atypia and eosinophilic cytoplasm. Neuroendocrine differentiation was confirmed by immunohistochemical positivity for CD56, synaptophysin, and chromogranin A. Immunohistochemistry also confirmed the absence of myoepithelial cells around the tumor cell nests. Therefore, a diagnosis of invasive SPC was made. Additionally, tumor cell deposits in the intramammary and axillary lymph nodes were identified, and these deposits had the same histological characteristics as the invasive SPC of the nipple. The invasiveness of SPC can be difficult to determine. However, the tumor cell nests in the current case exhibited a retraction artifact, which is known to be associated with invasive carcinoma and a poor prognosis, as well as morphological patterns that have been previously identified as characteristic of invasive SPC. Although SPC is widely recognized as having a favorable outcome, the existence of exceptionally aggressive cases occurring in the nipple must be recognized. Additional cases of invasive SPC of the nipple are needed to analyze the clinicopathological correlation.

[A Case of Invasive Ductal Carcinoma with Paget's Disease of the Breast].

Paget's disease of the breast is usually localized in the nipple epidermis and lactiferous duct located near the nipple. Here, we report a rare case of synchronous breast carcinoma with Paget's disease and invasive ductal carcinoma. A 50-year-old woman was admitted to our hospital because of abnormalities in screening mammography findings. Ultrasonography(US) findings showed a 2.4×1.3×1.6 cm sized hypoechoic lesion in a region of the left mammary gland. Computed tomography (CT)findings did not reveal distant metastasis. Magnetic resonance imaging(MRI)revealed an approximately 2.2 cm sized irregular tumor. The pretreatment diagnosis was left non-invasive ductal carcinoma(cTisN0M0, Stage 0), and surgery was performed. The tumor was found in the range of 40.2×15.0 mm, many of which were breast ductal growth. An infiltrated image was confirmed at multiple sites, but the maximum size was 2.5×1.5 mm. Pathological findings of the main lesion revealed papillotubular carcinoma. Agglomeration of heterotypic cells with abundant cytoplasm was observed in the epidermis of the papilla. The final diagnosis was invasive breast cancer(pT1aN3M0, Stage Ⅲc, Luminal HER2)coexisting with Paget's disease.

Solid papillary carcinoma of the breast with an associated Cytokeratin 7- negative Paget's disease of the nipple. Report of a first case.

Solid papillary carcinoma (SPC) is a rare neoplasm of the breast showing a distinct morphology, neuroendocrine differentiation and should be divided into invasive and in situ subtype according to the current 2012 WHO classification of breast tumors. Here, we describe a case of a pure SPC, invasive-type, in a 31-years old female with an associated mammary Paget's disease (MPD) of the nipple showing a rare, CK7-negative immune phenotype, which has not been reported so far. This unusual differential diagnosis should be added to the rare condition of CK7-negative Paget's disease of the breast and complement a new feature to the characterization of SPC.

Clinical and Sonographic Features of Mammary Paget's Disease.

Objective To summarize the clinical and sonographic features of mammary Paget's disease (MPD). Methods Totally 34 female patients with pathologically confirmed MPD were retrospectively recruited. According to diagnosis,the clinical data and sonographic findings were reviewed and analyzed. Results Among these 34 patients,7 patients had single MPD; in the remaining 27 patients,16 were accompanied by invasive ductal carcinoma (IDC),7 by ductal carcinoma in situ (DCIS),3 by both IDC and DCIS,and 1 by other pathologic types of carcinoma. Twenty-four patients presented with typical clinical features of MPD,whereas 10 patients had no typical features of MPD. Among the 24 patients with typical clinical features,ultrasound examinations showed that 11 had echoic abnormality in nipple-areolar complex,14 had lesions of breast,and 14 had microcalcifications. Among the 10 patients without typical clinical features,ultrasound examinations revealed echoic abnormality in nipple-areolar complex in 3 cases,breast lesions in 9 cases,and microcalcifications in 6 cases. Among the 14 patients with echoic abnormality in nipple-areolar complex,all of them presented as rich blood flow in nipple detected by color Doppler ultrasonography. Conclusions The main clinical feature of MPD is abnormalities in nipple-areolar complex. Sonography can recognize the echoic abnormalities of nipple and lesions of breast. Sonographer should be careful of the nipple-areolar complex when mass is found in breast.

Enlightening the Pink: Use of Confocal Microscopy in Pink Lesions.

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.