Young mammary Paget's disease patients with underlying breast invasive ductal carcinoma: clinicopathological features and prognosis / 中华肿瘤杂志

Objective: To investigate the clinicopathological factors and prognostic status of young Mammary Paget's disease (MPD) patients with invasive ductal carcinoma (IDC). Methods: In this study, we defined the age at diagnosis below 40 years old as young patients, and retrospectively analyzed data from 123 MPD-IDC patients who were admitted at the Cancer Hospital Chinese Academy of Medical Sciences from June 2002 to February 2019. Patients were divided into the young group (≤40 years old, 15 cases) and the old group (>40 years old, 108 cases) according to the age of onset, and the clinicopathological characteristics and prognosis of the two groups were compared. Cox regression model analysis was used to analyze the prognosis influencing factors. Results: The proportions of patients in the young group with non-menopausal, axillary lymph node metastasis, and Ki-67 index ≥15% were 93.3% (14/15), 73.3% (11/15), and 86.7% (13/15), respectively, which were higher than those in the old group [45.4% (49/108), 39.8%(43/108), and 60.2% (65/108), respectively] , with statistically significant differences (P<0.05). At an average follow-up of 63.2 months, patients in the young group had a significantly shorter disease-free survival (DFS) compared with that of the old group (P=0.012), while the difference in overall survival (OS) between the two groups was not statistically significant (P=0.161). Multifactorial Cox regression analysis showed that axillary lymph node status was an independent influencing factor on OS (HR=3.339, 95% CI: 1.121-9.943) in patients with MPD-IDC, while age was not. Conclusion: Compared with the old group, young patients with MPD-IDC have a higher incidence of axillary lymph node metastasis, high Ki-67 expression, and a shorter DFS, but age is not an independent influencing factor on DFS or OS in patients with MPD-IDC.

Enfermedad de Paget mamaria: revisión de la literatura / Paget's disease of the breast: review of the literature

Resumen La enfermedad de Paget mamaria es una neoplasia infrecuente de la mama, muchas veces de difícil diagnóstico y mal pronóstico. El objetivo es revisar los aspectos fundamentales clínicos, diagnósticos, así como los avances más recientes en su tratamiento. Se realizó una búsqueda bibliográfica a través de PUBMED, GOOGLE SCHOLAR y UPTODATE, de los últimos 20 años. Fueron seleccionados e incluidos en la revisión 38 artículos. La enfermedad de Paget mamaria es una neoplasia maligna infrecuente del complejo areola pezón de características inflamatorias al examen físico, asociado en un 85% de los casos a un cáncer de mama subyacente, en el 50% de los casos no identificable al examen físico, de difícil diagnóstico, cuya sospecha clínica se confirma con imágenes, biopsia e inmunohistoquímica. El tratamiento es mastectomía versus cirugía conservadora y radioterapia. Un mal enfoque clínico, retrasa el diagnóstico y empeora la sobrevida. MÉD.UIS.2021;34(1): 45-53.

Abstract Paget's disease of the breast is an uncommon neoplasm of the breast, many times difficult to diagnose and of poor prognosis. The objective is to review the fundamental clinical and diagnostic aspects, as well as the most recent advances in its treatment. A bibliographic search of the last 20 years was carried out through PUBMED, GOOGLE SCHOLAR and UPTODATE. 38 articles were selected and included in the review. Mammary paget disease is a rare malignant neoplasm of the nipple areola complex with inflammatory features on physical examination, associated in 85% of cases with underlying breast cancer, in 50% of cases not recognizable on physical exam, difficult to diagnose, whose clinical suspicion is confirmed by imaging, biopsy and immunohistochemistry. Treatment is mastectomy versus conservative surgery and radiotherapy. A poor clinical approach delays the diagnosis and worsens survival rate. MÉD.UIS.2021;34(1): 45-53.

Enfermedad de Paget. A propósito de un caso / Paget's disease. About a case / Doença de Paget. Sobre um caso

RESUMEN La enfermedad de Paget se define como una lesión neoplásica del epitelio escamoso de la piel de areola y/o pezón, que presenta unas células de citoplasma amplio y claro con nucléolos aumentados, núcleo atípico desplazado, y que están situadas en la epidermis a lo largo de la membrana basal, que la caracterizan y se conocen como células de Paget. Clínicamente se manifiesta como una lesión eccematosa o erosiva que no responde al tratamiento convencional. Se realiza la presentación de una paciente con esta enfermedad.

ABSTRACT Paget's disease is defined as a neoplastic lesion of the squamous epithelium of the areola and / or nipple skin, which has large and clear cytoplasm cells with enlarged nucleoli, displaced atypical nucleus, and that are located in the epidermis along of the basement membrane, which characterize it and are known as Paget cells. Clinically it manifests as an eczematous or erosive lesion that does not respond to conventional treatment. The presentation of a patient with this disease is made.

RESUMO A doença de Paget é definida como uma lesão neoplásica do epitélio escamoso da aréola e / ou pele do mamilo, que possui células grandes e claras de citoplasma com nucléolos aumentados, núcleo atípico deslocado e localizadas na epiderme ao longo da membrana basal, que a caracteriza e é conhecida como células de Paget. Clinicamente, manifesta-se como uma lesão eczematosa ou erosiva que não responde ao tratamento convencional. A apresentação de um paciente com esta doença é feita.

Mammary Paget's Disease of the Nipple: Relatively Common but Still Unknown to Many / 가정의학회지

Mammary Paget's disease is clinically defined as skin inflammation of the nipple area and is an adenocarcinoma of the epidermis of the nipple. The pathogenesis of mammary Paget's disease is relatively unknown; nonetheless, there are two popular theories that support the underlying carcinoma and de novo carcinogenesis. For the attending medical practitioner, mammary Paget's disease poses a diagnostic and therapeutic dilemma, especially in the absence of a clinically palpable breast mass. We report a rare case of a 48-year-old Malay woman who presented at Hospital Universiti Sains Malaysia, Kelantan, Malaysia with the symptom of skin erosion on the left nipple and unresponsiveness to multiple topical treatments. A full evaluation and assessment of the patient were conducted, and mammary Paget's disease was diagnosed.

Pigmented Mammary Paget Disease Misdiagnosed as Malignant Melanoma

Pigmented mammary Paget disease is a very rare clinicopathologic variant of mammary Paget disease. Diagnosis is often difficult because its clinical and histological features are very similar to those of malignant melanoma. Herein, we report a case of pigmented mammary Paget disease misdiagnosed as malignant melanoma.

Pigmented Mammary Paget Disease with Reticulated Features: A Rare Variant of Mammary Paget Disease

Pigmented mammary Paget disease (PMPD) is a rare subtype of mammary Paget disease that presents as a hyperpigmented patch or plaque over the areola and nipple. We herein report on an unusual case of PMPD with reticulated features in a 98-year-old female. The histology showed intraepidermal pagetoid cells containing melanin pigments without any underlying intraductal carcinoma.

Enfermedad de paget perianal / Extra mammary paget disease: report of one case

Extra mammary Paget disease is rare and mostly affects perianal and vulvar zones, in patients of both sexes and from the sixth decade of life. We report a 66 years old woman with a vulvar Paget disease, subjected to a vulvectomy. Two years later, a pruriginous and erythematous plaque appeared in the perianal region. The pathological study informed an extra mammary Paget disease. The patient was subjected to a perianal excision and the correction of the surgical defect in a second intervention. After one year of follow up, there is no evidence of disease relapse.

La enfermedad de Paget de localización extramamaria (EPEM) es una patología infrecuente que afecta preferentemente a zonas perianal y vulvar en pacientes de ambos sexos y desde la sexta década de vida. Presentamos un caso clínico tratado en conjunto por los Servicios de Cirugía General, Cirugía Plástica, Ginecología y Anatomía Patológica del Hospital Universitario de Getafe.

Pigmented Paget's Disease: A Rare Clinical Manifestation of Recurred Breast Carcinoma / 대한피부과학회지

Pigmented mammary Paget's disease is a rare variant of mammary Paget's disease, and this mimics malignant melanoma both clinically and histopathologically. Two women aged 48 and 44 years old, respectively, presented with 2-cm pigmented patches and 1-cm pigmented erosive crusted papules without discharge or palpable masses, respectively. They had previously undergone breast-conserving surgery following the diagnosis of intraductal carcinoma of the breast. Physical examinations and imaging studies revealed no evidence of the recurrence of breast cancer. However, histopathological and immunohistochemical analyses demonstrated the presence of 1) Paget's cells in the epidermis and these cells contained melanin within their cytoplasm and 2) numerous melanophages in the upper dermis. We diagnosed the two patients with pigmented Paget's disease. Subsequent pathological analyses revealed recurrent intraductal carcinoma. We recommend that skin biopsies should be taken from patients presenting with pigmented lesions of the nipple, who have previously undergone breast-conserving treatment, to test the possibility of cancer recurrence.

A Case of Pigmented Mammary Paget's Disease

Pigmented mammary Paget's disease is a uncommon clinicopathologic variant of mammary Paget's disease, and this mimics malignant melanoma both clinically and histopathologically. Herein, we report on a rare case of pigmented mammary Paget's disease. An 81-year-old woman presented with 2.5x1 cm sized, red and brown, eczematous plaque on her right areola, and she'd had this lesion for 3 years. Histopathology showed large, atypical cells with large nuclei and abundant pale cytoplasm throughout the epidermis. Dispersed melanocytes were noted in the epidermis and some of the Paget's cells contained melanin within their cytoplasm. Immunohistochemical studies demonstrated that the intraepidermal pagetoid cells were positive for cytokeratin 7; in contrast, they were negative for S-100, Periodic-acid Schiff (PAS), Alcian blue at PH 2.5, HMB-45 and carninoembryonic antigen (CEA). We recommend that pigmented mammary Paget's disease should be included in the differential diagnosis of pigmented lesions on the nipple.