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Paget's Disease of the Breast

Paget’s disease is a type of breast cancer that occurs in the ducts adjacent to the nipple and areola and spreads to the skin of the nipple and the areola. It accounts for less than 2 percent of all breast cancer cases, predominantly in women but with rare cases in men. Symptoms can be similar to eczema. Paget’s disease is usually associated with ductal carcinoma in situ (DCIS), limited to the nipple and areola area of the breast.

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This list of publications is regularly updated (Source: PubMed).

Fernandez-Flores A, Eraña I, Cuevas J
"Extramammary-Type" Paget Disease of the Breast.
Am J Dermatopathol. 2018; 40(10):711-720 [PubMed] Related Publications
BACKGROUND: Mammary Paget disease and extramammary Paget disease (EMPD) have different prognoses. Because they are indistinguishable on histopathological grounds, they must be distinguished on a topographical basis.
OBJECTIVE: To study cases of Paget disease of the breast, which show similarities to EMPD.
METHODS: Cases were selected by 2 different approaches: (1) those with an exceptionally good evolution and no evidence of subjacent tumor and (2) those expressing MUC5AC.
RESULTS: Five cases were collected. All cases showed an indolent behavior with a known long clinical history in 2 cases (9 and 25 years, respectively) and a long follow-up in all cases but one (4-8 years). In all cases but one, surgery was performed, and no parenchymal tumor was found (either intraductal or infiltrating). All cases expressed cytokeratin 7 and MUC5AC without expression of MUC2, S100, or p40.
LIMITATIONS: The short number of cases is a limitation of this study. In addition, case 5 is recent, so we have a very short follow-up.
CONCLUSIONS: Some cases of mammary Paget disease behave like EMPD with slow progression and with no underlying associated tumor. Immunoexpression of MUC5AC might be a clue to identify them.

Tjarks BJ, Kerkvliet AM, Jassim AD
Sebaceous Carcinoma in Situ Masquerading Clinically and Histologically as Paget Disease of the Breast.
S D Med. 2018; 71(8):350-353 [PubMed] Related Publications
Sebaceous carcinoma in situ is a poorly understood and ill-defined entity. In situ sebaceous carcinoma exhibits a similar location pattern to its invasive counterpart in that most commonly has a periorbital distribution. Review of the literature found only seven cases of extraocular sebaceous carcinoma in situ. We present a unique and challenging case of sebaceous carcinoma in situ masquerading both clinically and histologically as Paget's disease of the breast. A 61-year-old female presented to her dermatologist complaining of a 6 mm erythematous waxy papule on her medial right breast. The patient's past medical history was significant for Muir-Torre syndrome. Clinically, the differential diagnosis included Paget disease of the breast, squamous cell carcinoma, and sebaceous carcinoma. A shave biopsy revealed an atypical proliferation of large single cells limited to the epidermis infiltrating in a pagetoid pattern, as well as cohesive nests of round neoplastic cells with mild nuclear atypia, prominent nucleoli, and vacuolated cytoplasm. Histologically, the differential diagnosis included Paget's disease of the breast, squamous cell carcinoma in situ, melanoma in situ, and sebaceous carcinoma in situ. A battery of immunohistochemical stains was performed including CK7, EMA, CAM5.2, CK20, and MART-1. The lesional cells were positive for adipophilin, factor XIIIa, CK7, and EMA and were negative for CAM5.2, CK20, and MART-1 supporting a diagnosis of sebaceous carcinoma in situ. Multiple deeper sections were examined and invasion beyond the epidermis was not identified. This case adds to the paucity of information available regarding extraocular sebaceous carcinoma in situ and warns clinicians of this potential diagnostic pitfall especially in patients with Muir-Torre syndrome.

Karpathiou G, Chauleur C, Hathroubi S, et al.
Expression of CD3, PD-L1 and CTLA-4 in mammary and extra-mammary Paget disease.
Cancer Immunol Immunother. 2018; 67(8):1297-1303 [PubMed] Related Publications
BACKGROUND: Mammary and extra-mammary Paget disease is a rare form of intra-epithelial glandular neoplasm which is characteristically recurrent and necessitates multiple excisions that have an important impact on morbidity. Local immuno-modulating treatments have been applied with promising results, but the local immune markers of Paget disease have not been studied.
AIM OF THE STUDY: To investigate the local immune micro-environment of Paget disease.
MATERIALS AND METHODS: Sixty-four specimens from 41 patients, including cases with multiple recurrences and underlying primary neoplasm, have been studied for their expression of CD3, PD-L1 and CTLA-4.
RESULTS: Nineteen cases were mammary; 22 were extra-mammary and involved the vulva, the anus, the inguinal region and the lower extremity. PD-L1 was not expressed by any neoplastic lesion or the associated lymphocytes. CTLA-4 expression was found in nine cases. Higher stromal CD3 expression and moderate levels of intra-epithelial CD3 expression were present in most cases. Biopsies, subsequent excision specimens and recurrences showed the same immunohistochemical profile of CD3 and PD-L1, although there were different levels of CTLA-4 in a few cases. The underlying lesions in mammary Paget disease showed the same immunohistochemical profile as the intra-epithelial neoplastic cells. The expression of the markers did not correlate with age, sex, localization or recurrence.
CONCLUSION: Paget disease is characterized by an intense lymphocytic response, devoid of the immune-suppressive impact of the PD-L1 pathway, but with occasional CTLA-4 expression.

Ogata H, Mitsuzuka Y, Honma N, et al.
Sonographic visualization of nipple blood flow can help differentiate Paget disease from benign eczematous nipple lesions.
PLoS One. 2018; 13(5):e0197156 [PubMed] Free Access to Full Article Related Publications
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.

Mai R, Zhou S, Zhou S, et al.
Transcriptome analyses reveal FOXA1 dysregulation in mammary and extramammary Paget's disease.
Hum Pathol. 2018; 77:152-158 [PubMed] Related Publications
Paget's disease (PD) is an uncommon intraepithelial adenocarcinoma with unknown pathogenesis. There are two anatomic subtypes: mammary (MPD) and extramammary (EMPD). Little is known about their molecular characteristics. Our objective was to discover novel molecular markers for PD and its subtypes. In the discovery phase, we used transcriptome analyses to uncover the most differentially expressed genes and pathways in EMPD biopsies compared with normal skin. In the validation phase, we performed immunohistochemistry analyses on the most promising marker (FOXA1) and other markers selected from a literature review (GATA3, estrogen receptor [ER], and androgen receptor [AR]) on independent biopsies of MPD (n = 86), EMPD (n = 59), and normal skin (n = 21). Transcriptome analyses revealed 210 genes differentially expressed more than 10-fold between EMPD and normal skin. These genes are involved in mammary and sweat gland development (FOXA1) and immune regulation, as well as epidermal differentiation. Immunohistochemistry staining revealed that FOXA1 was positive in 88% of both MPD and EMPD, whereas GATA3 was positive in 67% of MPD and 77% of EMPD, and ER was positive in 9% of MPD and 19% of EMPD. Finally, AR was positive in 33% of PD and 54% of EMPD. Mammary Paget's disease and EMPD share dysregulation of the glandular developmental regulator gene FOXA1, suggesting similarity in cell-specific transcriptional regulation. Further, FOXA1 may be a useful molecular target for developing PD therapies.

Domoto H, Watanabe A, Sakata M, et al.
Invasive Solid Papillary Carcinoma of the Nipple With Pagetoid Extension and Nodal Metastasis.
Int J Surg Pathol. 2018; 26(6):573-577 [PubMed] Related Publications
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.

Lim GH, Chinthala JP, Gudi MA
Invasive papillary breast cancer with Paget's disease: first reported association and literature review.
BMJ Case Rep. 2018; 2018 [PubMed] Related Publications
Invasive papillary carcinoma of the breast and Paget's disease are both rare entities. We report the first known case of invasive papillary carcinoma associated with Paget's disease in a woman who presented clinically with a nipple mass. While invasive papillary carcinoma has been reported to have an indolent course without need for overtreatment, our patient had unusual unfavourable histological features. Literature review of this rare subtype was performed.

Rupp NJ, Rodewald AK, Chiesa F, Varga Z
Solid papillary carcinoma of the breast with an associated Cytokeratin 7- negative Paget's disease of the nipple. Report of a first case.
Breast J. 2018; 24(4):637-641 [PubMed] Related Publications
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.

Baumgarten HD, Showalter SL, Rochman CM, Keim-Malpass J
Erythematous, friable nipple with loss of protrusion · history of breastfeeding · Dx?
J Fam Pract. 2017; 66(12):758-760 [PubMed] Related Publications
A 34-year-old healthy woman presented to the breast surgical oncology clinic with skin changes to her left nipple after being referred by her primary care provider. She attributed the skin changes to shearing from breastfeeding her third child 5 years earlier. Physical examination revealed an erythematous and friable nipple with loss of protrusion. The patient reported routine bleeding from her nipple, but said the skin changes had remained stable and denied any breast masses. The patient's last mammogram was 2.5 years earlier and had only been remarkable for bilateral benign calcifications.

Wei Y, Zhu Q, Li J, Jiang Y
Clinical and Sonographic Features of Mammary Paget's Disease.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2017; 39(3):396-400 [PubMed] Related Publications
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.

Broecker JS, Sewell CW, Shehata BM, et al.
An atypical presentation of Paget's Disease of the breast without nipple involvement: Case report and review of the literature.
Pathol Res Pract. 2017; 213(11):1454-1456 [PubMed] Related Publications
We present a case of a 63 year-old Caucasian female who developed a right breast skin lesion discrete from the nipple that was subsequently diagnosed as Paget's Disease of the breast (PDB). Imaging did not reveal an underlying breast cancer or involvement of the nipple. The patient underwent a segmental mastectomy preserving the nipple and final pathology demonstrated residual Paget's disease of the skin and did not reveal any additional underlying breast carcinoma. To our knowledge, this case represents the first reported diagnosed case of isolated PDB without nipple involvement.

Lopes S, Vide J, Moreira E, et al.
Paget disease of the male breast.
Dermatol Online J. 2017; 23(4) [PubMed] Related Publications
Paget disease of the breast is an uncommon tumor of the nipple-areola complex that usually presents as an erythematous and erosive lesion. We report the case of a 61-year-old man that presented with a three-year history of an erythematous lesion of the right areola, first treated with topical corticosteroids without benefit. He was then referred to our dermatology department and the clinical suspicion of Paget disease was considered. The diagnosis was later confirmed by biopsy. This case report highlights the importance of clinical recognition of this entity along with other diseases that mimic these skin changes in order to allow earlier diagnosis and proper follow-up.

Elbendary A, Xue R, Valdebran M, et al.
Diagnostic Criteria in Intraepithelial Pagetoid Neoplasms: A Histopathologic Study and Evaluation of Select Features in Paget Disease, Bowen Disease, and Melanoma In Situ.
Am J Dermatopathol. 2017; 39(6):419-427 [PubMed] Related Publications
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.
RESULTS: Comparison between mammary Paget and extramammary Paget disease showed no significant differences in the features studied. Crushing of basal keratinocytes, presence of atypical cells in the corneum, and presence of large cells with amphophilic cytoplasm were significantly noted in Paget disease. Transition between the atypical clear cells and surrounding keratinocytes was absent in all cases of melanoma in situ and in 87 (60.4%) cases of Paget disease, but it was significantly associated with Bowen disease (98.6%). Dyskeratotic cells were significantly associated with Bowen disease cases.
CONCLUSION: Our study demonstrated a practical histologic approach to differentiate between intraepidermal pagetoid neoplasms. Careful histologic study of the proposed criteria may reduce reliance on immunohistochemical stains.

Wu Q, Ding X, Li J, et al.
Surgical treatment in Paget's disease with invasive ductal carcinoma: an observational study based on SEER.
Sci Rep. 2017; 7:45510 [PubMed] Free Access to Full Article Related Publications
The aim is to analyse the clinical presentation, treatment and outcomes in patients with Paget's disease with invasive ductal carcinoma (PD-IDC), with special emphasis on the role of surgical treatment. Using data obtained by the Surveillance, Epidemiology, and End Results (SEER) program from 2010-2013, we investigated the differences in characteristics, overall survival (OS), and breast cancer-specific mortality (BCSM) between patients with PD-IDC and those with invasive ductal carcinoma (IDC). Compared with IDC group, patients with PD-IDC had a better prognosis and lower mortality in adjusted analyses. In the multivariate analysis of cases with PD-IDC, history of ALND was significantly associated with OS while Her2 status were associated with BCSM. Further, subgroup analysis demonstrated no difference between surgical treatment subgroups for either OS or BCSM. The results demonstrated that PD-IDC appears to alter the association between prognosis and Her2 status. Meanwhile, breast-conserving surgery with radiotherapy may be a feasible treatment alternative and sentinel lymph node biopsy should be considered as an appropriate treatment for patients with PD-IDC.

Adams SJ, Kanthan R
Paget's disease of the male breast in the 21st century: A systematic review.
Breast. 2016; 29:14-23 [PubMed] Related Publications
Paget's disease of the breast is characterized by eczematous changes of the nipple-areolar complex and is associated with an underlying in situ or invasive breast carcinoma in most cases. Histologically, Paget's disease is identified by epithelial cells with abundant basophilic or amphophilic, finely granular cytoplasm with a large, centrally situated nucleus, most abundant in the lower epidermal layers. Due to the rarity of the condition among breast cancers, compounded by the rarity of breast cancer in men, understanding of the disease's presentation, course, and optimal treatment in men is largely derived from case reports and extrapolation of findings from studies in female patients. Paget's disease must be differentiated from other conditions including eczema, Bowen's disease, squamous cell carcinoma, and melanoma. Recognition of Paget's disease clinically and pathologically is critical as the superficial lesion may be the only sign of an underlying ductal carcinoma and its presence may be of prognostic significance. This article provides an update on cases of Paget's disease of the breast in men reported in the published literature together with a comprehensive analysis of the National Cancer Institute's Surveillance, Epidemiology, and End Results Data, 1973-2012. Current understanding and management of the disease in the context of male patients is reviewed. However, additional research is required to further understand the overall pathogenesis and molecular profile of Paget's disease to provide improved insight for personalized, precision-based therapeutic options.

Alessio C, Scali E, Manti F, et al.
An unusual case of mammary Paget’s disease in a woman with psoriasis.
J Biol Regul Homeost Agents. 2016 Apr-Jun; 30(2):589-92 [PubMed] Related Publications
Mammary Paget’s disease (MPD) is a malignant breast tumor, which is characterized by intraepidermal infiltration from malignant glandular epithelial cells. Often it may include an underlying ductal carcinoma in situ or an invasive ductal carcinoma. Clinically it appears as an erythematous patch, moist or crusted, with or without desquamation that in some cases becomes ulcerated, causing infiltration and inversion of the nipple. We report the clinical case of a 60-year-old woman, treated in our department for psoriasis, presenting with erythema of nipple and areola with nipple erosion, ulceration and poor secretion. Suspecting Paget’s disease of the nipple, radiological exams (mammography and breast MRI) were performed. A biopsy for histological examination was carried out and confirmed the diagnosis of mammary Paget’s disease. MPD is sometimes difficult to diagnose both clinically and radiologically, therefore it is important to distinguish from other conditions: in literature MPD is reported in differential diagnosis with psoriasis given its similar clinical features, and in some cases MPD has been treated with topical and systemic steroids due to a wrong diagnosis. However, the concomitance, in the same individual, of mammary Paget’s disease and psoriasis has never been described.

Ozerdem U, McNiff JM, Tavassoli FA
Cytokeratin 7-negative mammary Paget's disease: A diagnostic pitfall.
Pathol Res Pract. 2016; 212(4):279-81 [PubMed] Related Publications
Pathologists should be aware of the existence of a rare CK7-negative variant of breast carcinoma in general, and of Paget's disease in particular. Cytokeratin 7-negative Paget's disease and CK7-negative ductal intraepithelial neoplasia (ductal carcinoma in situ) present a major diagnostic challenge for pathologists since there is limited awareness of their existence. When there is classic Paget's morphology on H&E sections, GATA3 positivity should resolve any doubts about the diagnosis in the setting of a CK7-negative neoplastic cell population.

Vide J, César A, Rodrigues Pereira P, Azevdo F
Pigmented lesion of the nipple - a clinicopathological challenge.
Dermatol Online J. 2016; 22(10) [PubMed] Related Publications
Differential diagnoses of pigmented lesions of the nipple include melanocytic nevus, melanosis of the nipple, seborrheic keratosis, pigmented basal cell carcinoma, melanoma and Paget disease. The histologic exam with appropriate immunohistochemistry is a fundamental tool to achieve a correct diagnosis. We present a patient with a pigmented lesion of her right nipple revealing mammary Paget disease and elucidate diagnostic obstacles and prognostic importance of early breast cancer detection.

Saluja K, Sahoo S
Invasive Paget Disease of the Nipple of Luminal-B Subtype With Axillary Lymph Node Metastasis in a 60-Year-Old White Woman.
Lab Med. 2015; 46(4):332-7 [PubMed] Related Publications
Herein, we report a rare case of invasive Paget disease of the nipple with axillary-lymph-node metastasis in a 60-year-old white woman. The patient had intermittent, bloody nipple discharge without skin changes of the nipple-areolar region. We considered the clinical diagnosis of intraductal papilloma. A subareolar core biopsy revealed invasive ductal carcinoma in deep dermal tissue without the overlying epidermis biopsied. The patient underwent total mastectomy and axillary sentinel lymph-node biopsy that demonstrated invasive Paget disease of the nipple with 3.5-mm depth of invasion, ductal carcinoma in-situ in the underlying breast parenchyma, and macrometastasis (5.0 mm) in the sentinel lymph node. Prognostic marker studies of the metastatic site revealed a profile similar to that in the invasive mammary Paget disease (estrogen receptor [ER]+/progesterone receptor [PR]+/human epidermal growth factor receptor 2 [HER2]-/proliferation index [Ki-67] of 30%). The patient received adjuvant chemotherapy and experienced no disease recurrence at 20-months of follow-up. This case of luminal-B subtype invasive Paget disease as the source of regional metastasis is unique in the literature, to our knowledge.

Farouk O, Attia E, Roshdy S, et al.
The outcome of oncoplastic techniques in defect reconstruction after resection of central breast tumors.
World J Surg Oncol. 2015; 13:285 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Traditionally, conservative breast surgery was contraindicated in centrally located breast tumors, with total mastectomy as the treatment of choice. However, restorations of the central defects by the oncoplastic volume displacement or replacement techniques have been shown to be effective. The aim of the current study was to assess the surgical outcome of oncoplastic techniques after central breast tumors resection.
METHODS: Thirty patients with central breast cancer, including two patients with Paget disease, treated at the Oncology Center of Mansoura University (Egypt) between June 2011 and December 2014 were included in this study. The oncoplastic techniques performed were Grisotti advancement rotational flap in eight (26.7%) patients, classic skin-sparing mastectomy (SSM) with latissimus dorsi pedicled flap in 20 (66.7%) patients, and skin-reducing mastectomy (SRM) with latissimus dorsi pedicled flap using wise pattern inverted T incision in two (6.7%) patients. The choice of the oncoplastic techniques depends on the achievement of free safety margins, the breast volume, and its ptotic degree.
RESULTS: The median age was 40.5 years (range; 23-55). There were no major complications that require repeating the oncoplastic techniques. Recorded complications included wound dehiscence (4/30, 13.3%) donor site seroma (4/30, 13.3%), and surgical site infection (1/30, 3.3%). The 6-month subjective patient satisfaction was excellent in 21 (70%) patients, good in 6 (20%) patients, and fair in 3 (10%) patients. There was no episode of local recurrence or systemic metastasis after an average follow-up duration of 24 months (range; 6-42).
CONCLUSIONS: Restoring the central defect after resection of the central breast tumors can be safely achieved using oncoplastic procedures including the Grisotti technique or the design of SSM or SRM with immediate breast reconstruction. In our patients, these procedures yield a satisfactory esthetic outcome with lower morbidity.

Wong SM, Freedman RA, Sagara Y, et al.
The effect of Paget disease on axillary lymph node metastases and survival in invasive ductal carcinoma.
Cancer. 2015; 121(24):4333-40 [PubMed] Related Publications
BACKGROUND: The objective of this study was to examine the effect of Paget disease (PD) on axillary lymph node metastases and survival in patients who had concomitant invasive ductal carcinoma (PD-IDC).
METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to identify women who were diagnosed with PD-IDC from 2000 to 2011, comparing baseline demographic and tumor characteristics with those who were diagnosed with IDC alone during the same period. Multivariable logistic regression was used to examine the association of PD-IDC with axillary lymph node metastasis, and breast cancer-specific survival and overall survival were compared between the PD-IDC and IDC groups using the Kaplan-Meier method and Cox proportional hazards regression.
RESULTS: The study cohort included 1102 patients with PD-IDC and 302,242 controls with IDC alone. PD-IDC tumors were more likely to be centrally located (26.9% vs 5.5%; P < .001), high grade (63.5% vs 40.3%; P < .001), >2 cm in greatest dimension (47.1% vs 35.7%; P < .001), and estrogen/progesterone receptor-negative (45.2% vs 22.1%; P < .001). In adjusted analyses, patients with PD-IDC had higher odds of axillary lymph node metastasis (odds ratio, 1.83; P < .001). The unadjusted 10-year breast cancer-specific and overall survival rates were lower for the PD-IDC group compared with the IDC-alone group, although, after adjusting for disease stage, tumor characteristics, and local therapy, no significant differences in mortality risk were observed between the 2 groups (hazard ratio, 0.91; P = .24).
CONCLUSIONS: PD-IDC is associated with an increased risk of axillary lymph node metastasis, but not with inferior survival, compared with IDC alone after adjustment for other disease factors.

Szpor J, Polak K, Dyduch G, et al.
Pigmented Paget's disease of the nipple.
Pol J Pathol. 2015; 66(1):93-7 [PubMed] Related Publications
Pigmented Paget's disease of the nipple (PPD) is an uncommon variant of Paget's disease. An accumulation of melanin within the lesion imparts a brow color to the affected area, so the lesion might clinically as well as histologically mimic melanoma. We present a case of PPD in a 60-year-old woman.

Wong SM, Freedman RA, Stamell E, et al.
Modern Trends in the Surgical Management of Paget's Disease.
Ann Surg Oncol. 2015; 22(10):3308-16 [PubMed] Related Publications
PURPOSE: We examined the incidence and modern national trends in the management of Paget's disease (PD), including the use of breast-conserving surgery (BCS), mastectomy, axillary surgery, and receipt of radiotherapy.
METHODS: Using surveillance, epidemiology and end results (SEER) data, we identified 2631 patients diagnosed with PD during 2000-2011. Of these patients, 185 (7%) had PD of the nipple only, 953 (36.2%) had PD with ductal carcinoma in situ (PD-DCIS), and 1493 (56.7%) had PD with invasive ductal carcinoma (PD-IDC). Trends in age-adjusted incidence, primary surgery, sentinel lymph node biopsy (SLNB), and axillary lymph node dissection were examined. Multivariable logistic regression was used to evaluate factors associated with receipt of BCS and radiotherapy.
RESULTS: A decrease in the age-adjusted incidence of PD occurred from 2000 to 2011 (-4.3% per year, p < 0.05). The overall rates of mastectomy in the PD only, PD-DCIS, and PD-IDC groups were 47, 69, and 88.9%, respectively. Only in the PD-IDC group did the proportion of patients undergoing BCS increase significantly, from 8.5% in 2000 to 15.7% in 2011 (p = 0.01). Of those who underwent axillary surgery, the proportion of patients undergoing SLNB increased from 2000 to 2011. In adjusted analyses, Paget's subgroup, older age, central tumor location, low/intermediate grade, tumor size <2.0 cm, SEER region, and year of diagnosis after 2006 were significantly associated with receipt of BCS.
CONCLUSIONS: The incidence of Paget's disease has decreased over time while modern trends in local therapy suggest that BCS, SLNB, and adjuvant radiotherapy remain underutilized.

Helme S, Harvey K, Agrawal A
Breast-conserving surgery in patients with Paget's disease.
Br J Surg. 2015; 102(10):1167-74 [PubMed] Related Publications
BACKGROUND: Paget's disease of the breast is a rare condition that is associated with underlying breast cancer in the majority of patients. The conventional treatment for Paget's disease has been mastectomy, but there is an increasing trend to consider breast-conserving surgery (BCS) in selected patients. Owing to the uncommon nature of the disease, research studies tend to be small and retrospective. This systematic review presents the published evidence regarding BCS for patients with Paget's disease with a focus on patient selection and oncological safety.
METHODS: A search of Ovid and PubMed databases was conducted to identify all papers published regarding BCS for Paget's disease.
RESULTS: The search identified 172 papers of which 43 were clinically relevant. BCS is a safe alternative to mastectomy, provided a clear surgical margin is achieved and adjuvant radiotherapy used. However, patients with Paget's disease should be assumed to have underlying breast cancer, and these cancers tend to have poor biological profiles. When BCS is considered, careful preoperative investigation should be undertaken to identify the presence and extent of an underlying cancer. These cancers can be mammographically occult, multifocal or multicentric. Although the evidence is limited, there may be a role for MRI in selecting patients with Paget's disease for BCS.
CONCLUSION: Patients with Paget's disease are candidates for breast conservation with appropriate preoperative investigations. Oncological outcomes are equivalent to those of mastectomy if surgical margins are achieved and adjuvant radiotherapy is given.

Lopes Filho LL, Lopes IM, Lopes LR, et al.
Mammary and extramammary Paget's disease.
An Bras Dermatol. 2015 Mar-Apr; 90(2):225-31 [PubMed] Free Access to Full Article Related Publications
Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.

Napolitano L, Crowe D
Pigmented Mammary Paget Disease Mimicking Superficial Spreading Melanoma in an Elderly African-American Female.
J Cutan Med Surg. 2015 May-Jun; 19(3):313-6 [PubMed] Related Publications
BACKGROUND: Pigmented mammary Paget disease (PMPD) is a rare disease that may mimic cutaneous melanoma both in clinical presentation and on histology.
OBJECTIVE: The goal of this study was to discuss the clinical and histologic similarities between PMPD and cutaneous melanoma and how to differentiate between the two diseases.
METHODS: We describe an African-American patient with PMPD who was thought to have cutaneous melanoma on presentation. We describe the similarities of PMPD to cutaneous melanoma both clinically and on histology and discuss the methods of differentiation.
RESULTS: Clinical examination revealed a large pigmented patch of the left breast that appeared asymmetrical with irregular borders with a highly variable color pattern throughout. Histologic evaluation showed characteristics shared between PMPD and cutaneous melanoma. Immunohistochemical staining was needed for differentiation.
CONCLUSION: PMPD is a rare disease and is similar in clinical presentation and on histology to cutaneous melanoma. Immunohistochemical staining must be used to differentiate between the two diseases.

Alikanoglu AS, Yildirim M, Suren D, et al.
Expression of Cox-2 and Bcl-2 in Paget's disease of the breast.
Asian Pac J Cancer Prev. 2015; 16(3):1041-5 [PubMed] Related Publications
BACKGROUND: Paget's disease (PD) is a rare form of intraepithelial adenocarcinoma that involves breast and extramammarian tissues. It is often associated with ductal carcinoma in situ and/or invasive ductal cancer. Molecular pathways that play a role in development of Paget's disease are stil unclear. Expression patterns of Cox-2 and bcl-2 were therefore assessed.
MATERIALS AND METHODS: Patients with a histopathological diagnosis of Paget's disease were included in this study. Patient files were analysed retrospectively.
RESULTS: Invasive cancer was diagnosed in 35 (76.1%) of the patients, 7 (15.2%) had ductal carcinoma in situ and 4 (8.7%) patients had no associated neoplasm. Twenty four (52.2%) patients showed COX-2 expression in Paget cells whereas no expression was seen in 22 (47.8%) patients. No relation was found between COX-2 expression and the lesion underlying Paget's disease (p=0.518). Bcl-2 expression in Paget cells was found positive in 12 (26.1%) and negative in 27 (58,7%) cases. There was no relation between Bcl-2 expression and the lesion accompanying Paget's disease (p=0.412). No relation was observed between COX-2 expression and Bcl-2 expression (p=0.389).
CONCLUSIONS: In breast cancer, COX-2 expression is associated with poor prognostic factors. As COX-2 expression increases the tendency to metastasize also increases. In our study we found a significantly high COX-2 expression in Paget's disease of the breast. We suggest that COX-2 expression and inflammatory processes may play a role in pathogenesis of the Paget's disease of the breast.

Salemis NS
Florid papillomatosis of the nipple: a rare presentation and review of the literature.
Breast Dis. 2015; 35(2):153-6 [PubMed] Related Publications
Florid papillomatosis (FP) of the nipple, or nipple adenoma, is a rare benign proliferative lesion originating from the lactiferous ducts of the nipple. It most commonly affects women in their fourth and fifth decades of life. Clinically, in most cases FP presents with serous or serosanguinous nipple discharge while in many cases an erythematoid or eroded lesion, a nodule or a swelling of the nipple may coexist thus resembling Paget's disease. We describe a case of FP with a very unusual clinical presentation. Diagnostic evaluation and management of the patient are discussed along with a review of the relevant literature. We conclude that FP should be always considered in the differential diagnosis of patients presenting with a rapidly enlarging nipple nodule even in the absence of any skin lesions or nipple discharge. Early diagnosis and prompt treatment are essential. Simple complete excision in order to eliminate the risk of recurrence is the treatment of choice for FP. Although in rare cases a coincidental ipsilateral or contralateral breast cancer has been reported, FP is considered as an entirely benign clinical entity associated with excellent prognosis.

Lee HW, Kim TE, Cho SY, et al.
Invasive Paget disease of the breast: 20 years of experience at a single institution.
Hum Pathol. 2014; 45(12):2480-7 [PubMed] Related Publications
Mammary Paget disease with dermal invasion (invMPD) is rare, and its prognosis remains largely unknown. We reviewed MPD cases diagnosed at our institution and analyzed the clinicopathological characteristics of invMPD and non-invMPD to compare their incidences and outcomes. We retrospectively reviewed 205 cases of women diagnosed as having MPD between 1994 and 2013. Sixteen of 205 MPD cases (7.8%) had dermal invasion. Twelve of 16 invMPD cases had separate, underlying invasive breast carcinoma, and 3 invMPD cases had ductal carcinoma in situ. To exclude the influence of underlying disease on prognosis, we compared prognosis of invMPD with matched non-invMPD. The mean depth and extent of Paget cell invasion in invMPD cases were 0.637 and 1.268 mm, respectively. The horizontal extent of MPD was significantly larger in invMPD versus non-invMPD (mean, 14.31 mm versus 7.35 mm; P = .002). Distant metastasis and disease-related death were observed in 12.6% (24/189) and 12.1% (23/189) of non-invMPD patients, respectively, compared with 6.3% (1/16) and 6.3% (1/16) of invMPD patients; this difference was not significant (P = .7 and P = .7). Clinical outcomes of the invMPD patients were also not significantly different from the matched non-invMPD patients. In this study, MPD extent significantly correlated with MPD invasion. However, other clinicopathological parameters were not associated with dermal MPD invasion. Dermal MPD invasion was rare and did not predict regional lymph node metastasis or poor prognosis. The prognosis is usually similar for invMPD and non-invMPD, and MPD must be distinguished from locally advanced breast cancer presenting as satellite skin nodules.

Saeed D, Shousha S
Toker cells of the nipple are commonly associated with underlying sebaceous glands but not with lactiferous ducts.
J Clin Pathol. 2014; 67(11):1010-2 [PubMed] Related Publications
AIMS: Toker cells are clear cells present in the squamous epithelium of the nipple of some women. In contrast to squamous epithelium, they are cytokeratin 7 (CK7) positive. The origin of these cells is not completely understood. It has been suggested that they may represent abortive glands or migratory ductal cells; and may be precursors of Paget's disease of the nipple. Our aim was to investigate the incidence and distribution of Toker cells and their relationship with lactiferous ducts.
METHODS: We examined nipple sections from 100 consecutive mastectomies performed at Charing Cross hospital. New sections were stained for CK7 using the immunoperoxidase technique.
RESULTS: Toker cells were identified in 11 cases. They were always clustered within the squamous epithelium superficial to sebaceous glands with no relationship with lactiferous ducts. Two cases in the study had Paget's disease and these were not associated with underlying sebaceous glands.
CONCLUSIONS: This study suggests that Toker cells are more likely to be developmentally related to sebaceous glands rather than lactiferous ducts. This raises doubts about the presence of a relationship between Toker cells and the common forms of Paget's disease, as the latter are commonly seen in association with ductal carcinoma in situ (DCIS) involving underlying lactiferous ducts. Toker cells, however, may be related to a less common form of Paget's disease which is not associated with underlying DCIS.

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