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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).

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.

Oliveira A, Zalaudek I, Arzberger E, et al.
Reflectance Confocal Microscopy for Diagnosis of Mammary Paget's Disease.
Acta Derm Venereol. 2016; 96(7):980-982 [PubMed] Related Publications
is missing (Short communication).

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.

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.

Ozerdem U, Swistel A, Antonio LB, Hoda SA
Invasive Paget disease of the nipple: a brief review of the literature and report of the first case with axillary nodal metastases.
Int J Surg Pathol. 2014; 22(6):566-9 [PubMed] Related Publications
Although Paget disease of the nipple (PDN) is a well-established clinical and pathological neoplastic process, invasive PDN (IPDN) is a relatively newly described disease. The latter entity is characterized by invasive carcinoma that is localized to the nipple and is associated with PDN as well as with either intraductal and/or invasive carcinoma in the underlying breast. To our knowledge, only 17 cases of IPDN, all node negative, have been reported. Here, we report the case of a 68-year-old woman with invasive Paget disease of the left nipple. The patient had a history of intraductal carcinoma, treated by lumpectomy alone. She presented 6 years later with "eczematous" lesion of the ipsilateral nipple, a punch biopsy of which showed a superficially IPDN as well as conventional PDN. The subsequently performed wide excision of the nipple, areola, and underlying breast tissue showed the invasive carcinoma to span 0.6 cm. Then, 3 months later, the patient presented with ipsilateral palpable axillary lymphadenopathy. Axillary dissection revealed metastatic carcinoma in 7 of 19 lymph nodes. This case of IPDN not only represents the deepest extent of invasion reported thus far but also the only one known to be node positive.

Durkan B, Bresee C, Bose S, et al.
Paget's disease of the nipple with parenchymal ductal carcinoma in situ is associated with worse prognosis than Paget's disease alone.
Am Surg. 2013; 79(10):1009-12 [PubMed] Related Publications
Paget's disease of the nipple is often found in conjunction with underlying ductal carcinoma in situ (DCIS). In isolation, Paget's disease of the nipple, like DCIS, confers an excellent prognosis for survival. Our objective was to determine if Paget's disease identified with synchronous parenchymal DCIS has as favorable an outcome as Paget's disease alone. We analyzed a prospectively maintained pathology database and medical records to identify all patients diagnosed with Paget's disease of the nipple between June 1996 and December 2011. Overall survival was analyzed using Kaplan-Maier statistics and Cox proportional hazards modeling. Seventy-four patients were identified with Paget's disease: five (6%) with isolated Paget's of the nipple, 22 (30%) associated with parenchymal DCIS, and 47 (64%) associated with invasive cancer (± DCIS). Unexpectedly, patients with Paget's disease and DCIS had a worse prognosis than those with Paget's disease alone. Survival correlated with pathologic stage at diagnosis. Among the 16 deaths, median survival was 2.8 years (range, 0.1 to 15.2 years). Median follow-up for the entire cohort was 4.2 years (range, 0.1 to 15.2 years). Thus, Paget's disease with parenchymal DCIS may confer worse survival than isolated Paget's disease of the nipple, suggesting the difficulty of identifying invasive carcinoma within a background of DCIS.

Sandoval-Leon AC, Drews-Elger K, Gomez-Fernandez CR, et al.
Paget's disease of the nipple.
Breast Cancer Res Treat. 2013; 141(1):1-12 [PubMed] Related Publications
Paget's disease of the breast is a disorder of the nipple-areola complex that, while rare, is often associated with an underlying carcinoma. It is characterized by eczematoid changes of the nipple. Two theories have been proposed to explain the pathogenesis of Paget's disease. The Epidermotropic, which is the most accepted theory, suggests that Paget's cells originate from ductal cancer cells that had migrated from the underlying breast parenchyma. It is supported by the predominance of breast cancer markers found in Paget's disease. This article provides an overview of Paget's disease of the breast with special attention to immunohistochemistry and raises the question of new therapeutic approaches.

Kijima Y, Yoshinaka H, Hirata M, et al.
Oncoplastic breast surgery combining partial mastectomy with immediate breast reshaping using a keyhole-shaped skin glandular flap for Paget's disease.
Surg Today. 2014; 44(9):1783-8 [PubMed] Related Publications
Oncoplastic breast surgery (OBS), which combines the concepts of oncologic and plastic surgery, is becoming more common worldwide. We herein report the results of OBS in Japanese patients with Paget's disease. We performed OBS combining partial mastectomy with immediate breast reshaping using a keyhole-shaped skin glandular flap in two patients. In these two patients, who were diagnosed as having Paget's disease with a restricted intraductal component in the central area of their non-ptotic breast, we performed oncoplastic surgery combining partial mastectomy with immediate breast reshaping using a keyhole-shaped skin glandular flap. Neither of the two patients received contralateral surgery to produce symmetrical breasts. The observation period ranged from 6 to 12 months, and the bilateral breast volumes and inframammary lines were symmetric. OBS combining partial mastectomy with immediate breast reshaping using a keyhole-shaped skin glandular flap was successfully performed in two patients with Paget's disease.

Kryvenko ON, Yoon JY, Chitale DA, Lee MW
Prevalence of terminal duct lobular units and frequency of neoplastic involvement of the nipple in mastectomy.
Arch Pathol Lab Med. 2013; 137(7):955-60 [PubMed] Related Publications
CONTEXT: Breast cancer treatment has greatly evolved from radical mastectomy to more cosmetically acceptable and less-debilitating surgeries. Nipple-sparing mastectomy is increasingly done for both cancer treatment and risk reduction. The frequency of terminal duct lobular units (TDLUs) and occult neoplastic epithelial proliferation in grossly/clinically unremarkable nipples (GUNs) is not well investigated.
OBJECTIVE: To describe frequency of TDLUs and occult and overt neoplastic nipple involvement.
DESIGN: Nipples from 105 consecutive specimens (90 therapeutic, 15 prophylactic) were studied. Sixty-five nipples were entirely submitted to evaluate frequency of TDLUs; the rest had 1 vertical section submitted.
RESULTS: Terminal duct lobular unit was seen in 17 GUNs (26%). Six had TDLU in the base, 6 had it in the papilla, and 5 in both. Four GUNs showed lobular carcinoma in situ (1), Paget disease (1), and pagetoid extension of underlying malignancy (2). Grossly/clinically abnormal nipples had Paget disease (2), lymphovascular invasion (2), invasive carcinoma (4), and pagetoid extension (5). Involved nipples were closer to tumor (mean, 1.1 versus 3.2 cm, P < .001), had larger underlying tumors (mean, 4.3 versus 2.6 cm, P = .03) and of higher grade (P = .04), and more often had lymph node metastases (91% versus 44%, P = .007). No pathologic abnormalities were found in prophylactic mastectomy nipples.
CONCLUSIONS: Terminal duct lobular units were seen in 26% of nipples. They were frequently seen in the nipple papilla. Occult neoplastic epithelial proliferation was seen in 5% of grossly/clinically unremarkable therapeutic mastectomy nipples. Pagetoid extension was the dominant spread of underlying malignancy. Overall, the nipple was more often involved by larger and higher-grade tumors located closer to the nipple. All prophylactic mastectomies had unremarkable nipples. These findings should be considered while selecting patients for nipple-sparing mastectomy.

Bansal S, Sahoo B, Agarwal P, et al.
A rare presentation of mammary Paget's disease involving the entire breast in the absence of any underlying ductal malignancy.
Indian J Dermatol Venereol Leprol. 2013 Jul-Aug; 79(4):518-21 [PubMed] Related Publications
Paget's disease of the breast is an uncommon form of breast cancer presenting as an eczematous eruption over the nipple and/or areola. The diagnosis remains elusive with varied presentations, mimicking many benign skin diseases, the awareness of which is indispensable for diagnosis and minimizing morbidity. Most of the cases have an associated malignancy of the underlying breast tissue. There have been very few reports wherein the disease has occurred independent of any underlying malignancy. Since, the initial presentation is limited to skin; it is the dermatologist who plays a key role in making a diagnosis, thus, facilitating proper management. We report a rare presentation of mammary Paget's disease with a wide cutaneous spread probably attributed to a significantly delayed diagnosis without any associated underlying malignancy.

Crignis GS, Abreu Ld, Buçard AM, Barcaui CB
Polarized dermoscopy of mammary Paget disease.
An Bras Dermatol. 2013 Mar-Apr; 88(2):290-2 [PubMed] Free Access to Full Article Related Publications
Mammary Paget's disease is a rare intraepithelial adenocarcinoma, located on the nipple/areola complex, highly associated with breast cancer. Although the international literature emphasizes the dermatoscopic pattern of mammary Paget's disease pigmented variant, the authors describe the dermoscopic findings of classical Paget's disease and demonstrate the presence of chrysalis-like structures, criteria recently described in the literature and not yet reported in Paget's disease.

Rzaca M, Tarkowski R
Paget's disease of the nipple treated successfully with cryosurgery: a series of cases report.
Cryobiology. 2013; 67(1):30-3 [PubMed] Related Publications
INTRODUCTION: Paget's disease of the nipple is a rare form of breast malignancy. Underlying breast cancer, either in situ or invasive accompanies lesions of the nipple in most of the cases. The connection between both entities and their exact origin remains unclear. Nevertheless, underlying ductal breast cancer is often confined to the central, subareolar part of the breast. Radical mastectomy, although successful, seems to be too mutilating treatment in the era of breast sparing surgery. Studies describing breast conserving surgery performed without adjuvant treatment are rare and the patients not numerous. Due to low incidence of the disease, there are no randomised trials conducted which could show the optimal method of treatment.
MATERIALS AND METHODS: We performed cryosurgery of the affected nipple-areola complex (NAC) in treatment of six patients with Paget's disease of the nipple who presented general contraindications or lack of consent for surgery.
RESULTS: With a follow up ranging from 60 to 121 months (average 94) we obtained very good results in terms of disease specific survival: there were two cases of recurrent disease, confined to the scar and treated successfully with cryosurgery again; four patients are alive without disease. Death was not related to cancer in the remained two cases.
CONCLUSIONS: Cryotherapy is successful form of treatment of localised Paget's disease of the nipple--especially in face of contraindications for surgery or lack of patient's agreement for operational treatment.

Ling H, Hu X, Xu XL, et al.
Patients with nipple-areola Paget's disease and underlying invasive breast carcinoma have very poor survival: a matched cohort study.
PLoS One. 2013; 8(4):e61455 [PubMed] Free Access to Full Article Related Publications
Paget's disease (PD) of the breast is a rare disease. The survival rate of PD was reported to depend on the characteristics of the underlying carcinoma. This study aimed to investigate the characteristics and survival rate of PD patients with underlying invasive breast carcinoma (IBC). Fifty-two patients were diagnosed with PD and an associated IBC from 2001 to 2005 in Fudan University Shanghai Cancer Center. Twenty-four (46.2%) had no clinical manifestation of PD and were diagnosed unexpectedly by a histologic examination. The 52 patients were all recruited in this study as the PD group. They tended to have greater chances of lymph node involvement (53.8% vs. 35.7%), lower hormone receptor expression (34.6% vs. 69.7%), higher human epidermal growth factor receptor 2 (HER2) expression (76.9% vs. 21.3%), and worse survival (5-year relapse-free survival (RFS) 52.2% vs. 86.7%, P<0.01; breast cancer-specific overall survival (OS) 62.1% vs. 91.8%, P<0.01) when compared with patients diagnosed with IBC. A matched study was then performed to investigate whether the poor survival of patients in the PD group was due to the unfavorable prognosis of the underlying IBC. One hundred and fifty-six (3∶1 ratio of controls to PD patients) patients diagnosed with IBC only were recruited into the matched group. The match was conducted according to four variables: dimension of IBC, lymph node status, hormone receptor status and HER2 status. The 5-year RFS (52.2% vs. 81.4%, P<0.01) and OS (62.1% vs. 85.9%, P<0.01) were both lower for patients in the PD group than those in the matched group. Patients with PD and underlying IBC had poor survival. Their survival was worse than that of patients with IBC of similar stage and characteristics. For patients with no clinical PD manifestation who were histologically diagnosed as PD, survival might be worse compared to patients with clinically diagnosed PD.

Sanders MA, Dominici L, Denison C, et al.
Paget disease of the breast with invasion from nipple skin into the dermis: an unusual type of skin invasion not associated with an adverse outcome.
Arch Pathol Lab Med. 2013; 137(1):72-6 [PubMed] Related Publications
CONTEXT: Paget disease is an uncommon skin manifestation of breast cancer, associated with either invasive carcinoma or ductal carcinoma in situ in the underlying breast. In very rare cases, tumor cells within the epidermis invade through the basement membrane of the skin into the dermis.
OBJECTIVES: To identify a series of cases of Paget disease with direct dermal invasion and to investigate the clinicopathologic features and outcome.
DESIGN: Cases were identified during a 6-year period from the files of 2 hospitals. The clinical histories, imaging studies, and pathology reports were reviewed.
RESULTS: Seven patients were identified, 5 with microinvasion (<0.1 cm) and 2 with 0.2- or 0.3-cm invasive carcinomas in the dermis. No lymphovascular invasion was seen. Sentinel nodes were negative in 3 patients who underwent biopsy. Five patients were treated with breast conservation with radiation. Three patients were at high risk for breast cancer because of prior breast cancer, Li-Fraumeni syndrome, or radiation for Hodgkin disease. The latter 2 patients underwent bilateral mastectomies. Three patients received hormonal therapy and 1 oophorectomy. No patient received chemotherapy. At follow-ups ranging from 4 to 66 months (median, 20 months), there have been no recurrences.
CONCLUSIONS: Patients with direct dermal invasion from Paget disease had a favorable outcome during the available follow-up period. This type of dermal involvement must be distinguished from locally advanced invasive carcinomas with skin invasion classified as T4b in the American Joint Cancer Commission staging system, as cancers with other types of skin invasion are associated with a poor prognosis.

Psoma E, Nikolaidou O, Stavrogianni T, et al.
A rare case report of a primary large-cell neuroendocrine carcinoma of the breast with coexisting Paget disease.
Clin Imaging. 2012 Sep-Oct; 36(5):599-601 [PubMed] Related Publications
Neuroendocrine carcinomas are malignancies deriving from neuroendocrine cells existing in various sites of the body, most commonly in the lungs and gastrointestinal tract. Primary neuroendocrine carcinomas of the breast are extremely rare, and their diagnosis is confirmed by positive neuroendocrine markers. We describe a case of a 46-year-old woman with a palpable mass in her left breast for the previous 3 months. The tumor was resected and a primary large-cell neuroendocrine tumor of the breast was confirmed by histopathologic examination. Paget disease of the nipple was noted as well.

Zheng S, Song QK, Zhao L, et al.
Characteristics of mammary Paget's disease in China: a national-wide multicenter retrospective study during 1999-2008.
Asian Pac J Cancer Prev. 2012; 13(5):1887-93 [PubMed] Related Publications
The aim of this study was to detail characteristics of mammary Paget's disease (PD) representing the whole population in China. A total of 4211 female breast cancer inpatients at seven tertiary hospitals from seven representative geographical regions of China were collected randomly during 1999 to 2008. Data for demography, risk factors, diagnostic imaging test, physical examination and pathologic characters were surveyed and biomarker status was tested by immunohistochemistry. The differences of demography and risk factors between PD with breast cancer and other lesions were compared using Chi-square test or t-test, with attention to physical examination and pathological characters. The percentage of PD was 1.6% (68/4211) in all breast cancers. The mean age at diagnosis was 48.1, and 63.2% (43/68) patients were premenopausal. There is no difference in demography and risk factors between PD with breast cancer and other breast cancer (P>0.05). The main pattern of PD in physical exam and pathologic pattern were patients presenting with a palpable mass in breast (65/68, 95.6%) and PD with underlying invasive cancer (82.4%, 56/68) respectively. The rate of multifocal disease was 7.4% (5/68). PD with invasive breast cancer showed larger tumor size, more multifocal disease, lower ER and PR expression and higher HER2 overexpression than those in other invasive breast cancer (P<0.05). These results suggested that PD in China is a concomitant disease of breast cancer, and that PD with underlying invasive cancer has more multiple foci and more aggressive behavior compared with other breast invasive cancer. We address the urgent needs for establishing diagnostic and therapeutic guidelines for mammary PD in China.

Duan X, Sneige N, Gullett AE, et al.
Invasive paget disease of the breast: clinicopathologic study of an underrecognized entity in the breast.
Am J Surg Pathol. 2012; 36(9):1353-8 [PubMed] Related Publications
Mammary Paget disease (MPD) is considered an intraepidermal manifestation of an underlying mammary carcinoma. In contrast to extramammary Paget disease, invasion of mammary Paget cells into the dermis (invMPD) has not been reported, except for 2 cases described in Rosen's textbook. Our study was designed to identify the presence of dermal invasion of mammary Paget cells and characterize the associated clinicopathologic features. Slides from 146 MPD patients were retrieved. Six cases of invMPD were identified. One case of invMPD was not associated with an underlying breast cancer, 1 was associated with invasive ductal carcinoma (IDC), 1 with ductal carcinoma in situ (DCIS) with microinvasion, and 3 with DCIS only. The underlying breast carcinomas was separate from the area of invMPD. The depth of invasion, measured from the dermal-epidermal junction to the focus of deepest invasion, ranged from 0.02 to 0.9 mm. The horizontal extent ranged from 0.01 to 4.0 mm. Lymph node with isolated tumor clusters was present in case 1, which had no underlying carcinoma but had the greatest extent of invasion, and in case 3, which had DCIS with microinvasion. One patient (case 1) died of unrelated causes 2 years later, and the remaining patients were alive without disease at last follow-up. In summary, we describe 6 cases of MPD with invasion of Paget cells into the dermis and provide histopathologic criteria for the diagnosis of this rare and underrecognized entity. Further studies are required to determine whether invasion in MPD has clinical significance.

Marques-Costa JC, Cuzzi T, Carneiro S, et al.
Paget's disease of the breast.
Skinmed. 2012 May-Jun; 10(3):160-5; quiz 165 [PubMed] Related Publications
The initial presentation of Paget's disease of the breast is an eczematous lesion, similar to contact dermatitis, in the skin of the breast at the areola and/or nipple and is refractory to usual topical treatments. It is frequent among women, especially those in their 50s and 60s. Most cases are associated with an in situ or underlying invasive breast neoplasia. The histopathology is usually diagnostic, with immunohistochemistry useful for its differentiation from melanoma and other types of tumors. Treatment should always be surgical with invasive procedures for the effective and complete removal of the breast tumor.

Lohsiriwat V, Martella S, Rietjens M, et al.
Paget's disease as a local recurrence after nipple-sparing mastectomy: clinical presentation, treatment, outcome, and risk factor analysis.
Ann Surg Oncol. 2012; 19(6):1850-5 [PubMed] Related Publications
BACKGROUND: Paget's disease is a rare clinical and histological type of local recurrence (LR) after breast cancer treatment both in case of conservative surgery or nipple-sparing mastectomy (NSM) with or without intraoperative radiation.
METHODS: We performed an analysis of 861 NSM with electron beam intraoperative radiotherapy (ELIOT) patients treated at the European Institute of Oncology from 2002 to 2008, focused on Paget's disease local recurrence.
RESULTS: In 861 patients (713 invasive carcinoma and 148 intraepithelial neoplasia), there were 36 local recurrences (4.18%), and among these were 7 Paget's disease local recurrences (0.8%). Median follow-up was 50 months. Four cases presented with nipple areola complex (NAC) erosions, two crusted lesions, and one ulcerated NAC. The average latency period from the NSM to Paget's disease local recurrence is 32 months (range, 12-49). Complete NAC removal was performed in all seven recurrences. The average follow-up after NAC removal was 47.4 months (range, 20-78). We found neither locoregional relapse nor metastatic event in this group. All patients were alive without disease.
CONCLUSIONS: Paget's disease local recurrence can be found in a significant proportion after NSM. Any suspicious lesion on NAC requires prompt pathological confirmation. Primary carcinoma with ductal intraepithelial neoplasia or invasive ductal carcinoma with extensive in situ component, negative hormonal receptor, high pathological grade, overexpression of HER2/neu, and "HER2 positive (nonluminal)" subtype tend to be significantly associated with more Paget's disease local recurrence and should be followed carefully.

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