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RESUMEN La enfermedad de Paget de la mama se encuentra entre las llamadas formas especiales del cáncer de mama. Su incidencia es de solo el 1 al 1,5 % del total de los cánceres mamarios que se diagnostican. Fundamentalmente aparece en mujeres en la quinta o sexta décadas de la vida. Se considera extremadamente rara en su forma bilateral. Se presenta el caso de una paciente de 32 años de edad que acudió a consulta provincial de mastología por presentar lesiones escamosas en el complejo areola pezón de ambas mamas, más acentuadas en la derecha. Se le realizó biopsia con aspiración con aguja fina y se diagnostica enfermedad mamaria de Paget bilateral. La enfermedad de Paget de la mama es una enfermedad de rara aparición y en el caso que se presenta aparecen dos características pocos comunes, el tratarse de una paciente joven y la localización bilateral de la enfermedad, lo cual hace de más valor su publicación para conocimiento del personal médico dedicado a la atención de estas afecciones.
ABSTRACT Paget's disease of the breast is among the so-called special forms of breast cancer. Its incidence is only 1 to 1.5 % of all breast cancers that are diagnosed. It mainly appears in women in the fifth or sixth decade of life. It is considered extremely rare in its bilateral form. The case of a 32-years-old female patient of urban origin who attended the provincial mastology clinic for presenting scaly lesions in the nipple areola complex of both breasts, more accentuated on the right side. She underwent a fine needle aspiration biopsy and was diagnosed with bilateral Paget's disease of the breast. Paget's disease of the breast is a disease of rare appearance throughout the world and in the case that it occurs, two uncommon characteristics appear, the fact that it is a young patient and the bilateral location of the disease, which makes it more valuable its publication for the knowledge of medical personnel dedicated to the care of these conditions.
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Abstract Paget's disease is a rare disorder of the nipple and/or the areola that is characterized by an erythematosquamous lesion and is often associated with in situ or invasive breast carcinoma. The authors present an atypical, exuberant case that had evolved over eight years, emphasizing the importance of early diagnosis.
Subject(s)
Humans , Breast Neoplasms/diagnosis , Paget's Disease, Mammary , Thorax/diagnostic imaging , Ulcer , NipplesABSTRACT
Objective: To investigate the diagnostic value of high-frequency ultrasound for Paget disease of breast (PDB). Methods :High-frequency ultrasound data of 30 patients with pathology approved PDB were retrospectively analyzed, including 13 cases complicated with invasive ductal carcinoma (IDC). Ultrasonic appearances of PDB with or without IDC were compared. Results: Ultrasonic features of PDB included hypo-echogenicity in nipple, thicken areola skin, mammary nodules, calcification, dilated duct and enlarged axillary lymph nodes. Compared with PDB without IDC, PDB complicated with IDC showed more mammary nodules (P=0.001) and enlarged axillary lymph nodes (P=0.002). No significant difference of breast imaging reporting and data system (BI-RADS) classification, hypo-echogenicity in nipple, thicken areola skin, calcification nor dilated duct rate was found between groups (all P>0.05). Conclusion: PDB have certain ultrasonic characteristics. High-frequency ultrasound is helpful to diagnosis and assessment of PDB.
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Paget's disease is a rare entity that accounts for 1 to 3% of breast tumors. Occurrence in male patients is even less common. Literature has reported only 24 cases in the last 20 years. We described the case of a 62-year-old male patient that sought medical care due to erosion and eczema on left nipple. After skin biopsy, the clinical suspicion of Paget's disease was confirmed by histological and immunohistochemical studies, which enabled the proper surgical and oncological treatment
A Doença de Paget do mamilo é uma entidade rara, representando 1 a 3% dos carcinomas de mama. Sua presença em pacientes masculinos é ainda menos comum, com apenas 24 casos na literatura nos últimos 20 anos. Em nosso relato de caso, descrevemos um paciente masculino de 62 anos que procurou atendimento por erosão e eczema no mamilo esquerdo. Após biópsia de pele, a histologia e o estudo imuno-histoquímico confirmaram a suspeita clínica de Doença de Paget do mamilo, possibilitando o tratamento cirúrgico-oncológico adequado.
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Objective@#The aim of this study was to explore the clinicopathologic characteristics and prognostic factors of Paget′s disease.@*Methods@#137 patients with Paget′s disease of the breast who were diagnosed in Cancer Hospital Chinese Academy of Medical Sciences between January 2007 and May 2016 were identified and included in the study, including 134 females and 3 males.@*Results@#The average age at onset of the disease was 51.8 years(range, 27-78 years). Among the 137 patients, 7 cases were Paget′s disease without any other underlying malignancy, who were alive during the follow-up period. The other 130 patients were diagnosed with Paget′s disease and underlying ductal carcinoma in situ (DCIS) or (and) with an invasive carcinoma. The positive expression rates of estrogen receptor (ER), progesterone receptor (PR) and human epidermal growth factor receptor 2 (HER-2) were 29.2%(40/137), 38.0%(52/137)and 78.1%(107/137), respectively. The two-year and five-year overall survival were 99.0%, 96.0%, and disease-free survival of two-year and five-year were 97.6%, 92.8%, respectively for all 137 patients. On survival analysis, longer course of disease, HER-2 positivity, tumor stage(T1-T4), and axillary lymph node metastasis were significantly associated with overall survival.@*Conclusions@#Paget′s disease of the breast is a slowly progressive malignancy with good prognosis. A longer course of disease, HER-2 positivity, tumor stage, axillary lymph node metastasis and distant metastasis were significantly associated with poor prognosis.
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Objective: To evaluate the survival of a series of patients with Paget's disease of the breast. Methods: Observational, retrospective and descriptive study. Data were collected through electronic medical records; the following variables were obtained: age, tumor histology, tumor size, degree of differentiation, lymphatic invasion, vascular invasion, neural invasion, presence or not of potential involvement of axillary lymph nodes, immunohistochemical profile, treatments performed, recurrence, and follow-up. Results: Of the 301 cases of assisted breast cancer, six patients were identified with Paget's disease of the breast. The overall survival, with a mean follow-up of 54 months, was 100%. All individuals are free of disease activity. The most common histochemical profile was negative for estrogen and progesterone receptors, and positive for HER-2/neu. Axillary and/or distal metastatic involvement was not identified. Conclusions: Overall survival was 100%, with a mean follow-up of 54 months. This high rate is due to the absence of axillary and/or distal metastatic involvement in our series.
Objetivos: Avaliar a sobrevida de uma série de pacientes com doença de Paget na mama. Métodos: Estudo observacional, retrospectivo, descritivo. Foram coletados os dados através de prontuário eletrônico e obtidas as seguintes variáveis: idade, histologia do tumor, tamanho do tumor, grau de diferenciação, invasão linfática, invasão vascular, invasão neural, presença ou não de comprometimento de linfonodos axilares, perfil imunoistoquímico, tratamentos realizados, recidiva e seguimento. Resultados: Dos 301 casos de câncer de mama atendidos, foram identificadas 6 pacientes com doença de Paget na mama. A sobrevida global, com um seguimento médio de 54 meses, foi de 100%. Todas estão sem doença em atividade. O perfil histoquímico mais frequente foi negativo para receptores de estrógeno e de progesterona, e positivo para o HER-2/neu. Não foi identificado comprometimento metastático axilar e/ou a distância. Conclusões: A sobrevida global foi de 100%, com um seguimento médio de 54 meses. Essa alta taxa deve-se à ausência de comprometimento metastático axilar e/ou a distância em nossa série.
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La Enfermedad de Paget Mamaria (EPM), es una neoplasia infrecuente del complejo pezón/areola, que se presenta como expresión de un carcinoma intraductal adyacente. Presentamos dos casos de pacientes atendidas en nuestro servicio.
Mammary Paget's disease is a rare neoplasm of the nipple/areola area, which is presented as an expression of an adjacent intraductal carcinoma. We report two cases of patients treated in our service.
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Objetivo: Determinar a prevalência de doença de Paget da mama (DPM) entre os casos de carcinomas ductais diagnosticados em um centro universitário, entre 2003 e 2007, descrever as características clínicas e analisar a sobrevida desses casos. Métodos: Estudo de coorte retrospectiva, por meio da revisão de prontuários médicos. Foi realizada análise de frequência para todas as variáveis e utilizada curva de Kaplan-Meier para a representação da sobrevida global. Resultados: De 278 casos de carcinomas ductais de mama, houve 14 casos de DPM, determinando prevalência de 5,0%. Um caso foi excluído da análise por apresentar dados incompletos. A média de idade ao diagnóstico foi de 57,1 (±11,2) anos. Dos casos analisados, 11 (84,6%) apresentavam tumor palpável, e 9 (69,3%), lesão do complexo aréolo-papilar (CAP). Apenas um caso não foi submetido à mastectomia, por óbito durante quimioterapia neoadjuvante. Radioterapia foi realizada em 6 casos (46,2%), quimioterapia, em 11 casos (84,6%), e endocrinoterapia, em 6 casos (46,2%). A imunoistoquímica identificou 5 casos (38,5%) com expressão de receptores hormonais e 12 casos (92,3%) com superexpressão de HER2. A sobrevida global das pacientes foi de 61,5 (±13,4) meses e não houve recidiva local após um tempo médio de seguimento de 75,8 meses. Conclusão: Observou-se prevalência de DPM associada a carcinomas invasores com estádio clínico avançado, o que possivelmente ocasionou sobrevida global inferior à observada em estudos prévios para a região.
Objective: To determine the prevalence of Paget's disease of the breast (PD) among cases of ductal carcinomas diagnosed in a university hospital between 2003 and 2007; describe clinical characteristics and analyze the survival of these cases. Methods: Retrospective cohort study, by reviewing medical records. Frequency analysis was performed for all variables and used Kaplan-Meier curve for the representation of overall survival. Results: In 278 cases of breast ductal carcinoma, 14 cases were PD determining prevalence of 5.0%. A case was excluded from analysis because of incomplete data. The mean age at diagnosis was 57.1 (±11.2) years. Of the cases analyzed, 11 (84.6%) had palpable tumor and 9 (69.3%) presented lesion of the nipple-areola complex. Only one case was not submitted to mastectomy because she died during neoadjuvant chemotherapy. Radiotherapy was performed in six cases (46.2%); chemotherapy in eleven cases (84.6%); endocrinoterapia in six cases (46.2%). Immunohistochemistry identified five cases (38.5%) with expression of hormone receptors and twelve patients (92.3%) with overexpression of HER2. The overall survival of patients was 61.5 (±13.4) months, and there was no local recurrence after a mean follow-up of 75.8 months. Conclusion: There was a prevalence of Paget's disease of the breast associated with invasive carcinomas with advanced clinical stages; which possibly resulted in overall survival rate lower than that observed in other studies for the region.
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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.
Subject(s)
Female , Humans , Male , Breast Neoplasms/pathology , Paget Disease, Extramammary/pathology , Paget's Disease, Mammary/pathology , Skin Neoplasms/pathology , Breast Neoplasms/therapy , Carcinoma/pathology , Carcinoma/therapy , Diagnosis, Differential , Genital Neoplasms, Male/pathology , Genital Neoplasms, Male/therapy , Nipples/pathology , Paget Disease, Extramammary/therapy , Paget's Disease, Mammary/therapy , Skin Neoplasms/therapy , Vulvar Neoplasms/pathology , Vulvar Neoplasms/therapyABSTRACT
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. .
Doença de Paget mamária é considerada um adenocarcinoma intra-epitelial raro, localizado no complexo mamilo-aréola,com alta associação ao câncer de mama. Apesar da literatura mundial realçar o padrão dermatoscópico da doença de Paget mamária variante pigmentada os autores descrevem os achados dermatoscópicos da doença de Paget clássica realçando a presença das estruturas crisálida-símiles,critério recentemente descrito na literatura mundial e ainda não relatado na Doença de Paget. .
Subject(s)
Aged , Female , Humans , Breast Neoplasms/pathology , Dermoscopy/methods , Paget's Disease, Mammary/pathology , BiopsyABSTRACT
Technetium-99m methoxyisobutylisonitrile (99mTc-MIBI) uptake is known to be increased in breast cancer because of increased blood flow from angiogenesis and heightened metabolism. We performed a 99mTc-MIBI scan in a patient with mammary Paget's disease. The patient had underlying invasive cancer in the same side of the breast. 99mTc-MIBI scan exhibited a scintigraphic image of the uptake from the invasive cancer lesion located deeply in the breast toward the epidermis. 99mTc-MIBI showed an uptake in the deeply located invasive cancer lesion as well as nipple lesion. Especially, the delayed phase of Tc-MIBI scan demonstrated the tumor site more accurately. In conclusion, 99mTc-MIBI scan could be a useful adjunct to clinical decision making in the management of Paget's disease of the breast.