Doença de Paget da Mama: estudo retrospectivo de pacientes atendidos em Hospital Oncológico Terciário
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2023-05-26
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Universidade Estadual Paulista (Unesp)
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Introdução: Nas últimas décadas, profundas alterações ocorreram no tratamento do câncer de mama, com avaliação dos subtipos moleculares, estadiamento do ponto de vista prognóstico, maior utilização da ressonância magnética na extensão local da doença, utilização de Terapias-Alvo, além da consolidação da cirurgia oncoplástica. A Doença de Paget da Mama (DPM), neste contexto, é uma entidade rara, sendo apresentada com limitadas casuísticas. Inicialmente descrita como entidade clínica, porém tal fato não é unânime, constituindo uma doença multifocal/ multicêntrica. Objetivo: Avaliar o impacto das mudanças na mastologia em relação a DPM e a viabilidade da realização da cirurgia oncoplástica da mama (COM) nesta doença. Métodos: Estudo retrospectivo de pacientes atendidos em Hospital Oncológico Terciário. Na presença de alterações areolares, a DPM foi caracterizada como clínica e nos demais como subclínica/patológica, todas com diagnóstico anatomopatológico de Paget. Avaliou-se dados clínicos e prognósticos, comparando-se as diferentes formas clínicas. Foram descritas as situações relacionadas a realização das cirurgias e o seu impacto na recidiva local e sobrevida. Utilizou-se de estatísticas descritivas, comparação entre grupos (teste do qui-quadrado e Mann-Witney) e sobrevida (logrank; Kaplan & Meier). Avaliou-se a sobrevida específica por câncer. Resultados: 85 pacientes foram avaliadas. A forma clínica esteve presente em 58.8% das pacientes. Frente ao estadiamento 27.1% apresentavam doença estádio zero, sendo 92.9% doença multifocal/multicêntrica. A maioria das pacientes (83.5%) possuíam os subtipos moleculares HER 2 e Luminal HER 2. Pacientes com DPM forma clínica apresentaram uma maior taxa de doença "in situ" (p=0.028), sendo submetidas a tratamento conservador com maior frequência (p<0.001). Das 43 pacientes com doença invasora HER 2, a maior parte foi submetida a terapia antiHER. O seguimento médio foi de 71,2 ± 43,3 meses. A sobrevida atuarial específica por câncer aos 60 e 120 meses foi de 92,3% e 83,1%, respetivamente. Apesar de não significativo, a doença clínica/areolar apresentou melhor sobrevida (p=0.275). A terapia anti-HER (p=0.509) não influenciou a sobrevida, sendo o estádio clínico fator isolado (p=<0.001). Comparando-se as variáveis clínicas e patológicas a única que se associou a realização da COM foi a idade, em que pacientes na faixa de 40-49 estiveram associados a maior taxa de oncoplastia (p=0.002) e razão de risco de 3.22 [IC 3.39-184.50]. A COM não influenciou a recorrência local ou a sobrevida (p=0.132) Conclusão: A DPM é uma condição rara, associada à multifocalidade/ multicentricidade, hiperexpressão de HER 2 e diferença entre os grupos com doença clínica e outro com doença subclínica/patológica. A COM não influencia na recorrência local ou sobrevida. Necessitam-se mais estudos visando avaliar a doença clínica/areolar, e o impacto dos avanços da mastologia na DPM.
Introduction: In recent decades, profound changes have occurred in the treatment of breast cancer, with evaluation of molecular subtypes, staging from a prognostic point of view, greater use of magnetic resonance imaging in the local extension of the disease, use of Targeted Therapies, in addition to the consolidation of oncoplastic surgery. Paget's Disease of the Breast (MPD), in this context, is a rare entity, being presented with limited case series. Initially described as a clinical entity, however, this fact is not unanimous, constituting a multifocal/multicentric disease. Objective: To assess the impact of changes in mastology in relation to MPD and the feasibility of performing oncoplastic breast surgery (OBS) in this disease. Methods: Retrospective study of patients seen at a Tertiary Oncology Hospital. In the presence of areolar alterations, MPD was characterized as clinical and in the others as subclinical/pathological, all with Paget's anatomopathological diagnosis. Clinical and prognostic data were evaluated, comparing the different clinical forms. Situations related to the performance of surgeries and their impact on local recurrence and survival were described. Descriptive statistics, comparison between groups (chisquare and Mann-Witney test) and survival (log-rank; Kaplan & Meier) were used. Cancer-specific survival was evaluated. Results: 85 patients were evaluated. The clinical form was present in 58.8% of the patients. Regarding staging, 27.1% had stage zero disease, with 92.9% multifocal/ multicentric disease. Most patients (83.5%) had the molecular subtypes HER 2 and Luminal HER 2. Patients with clinical MPD had a higher rate of "in situ" disease (p=0.028), being submitted to conservative treatment more frequently (p <0.001). Of the 43 patients with HER 2 invasive disease, most underwent anti-HER therapy. Mean follow-up was 71.2 ± 43.3 months. The actuarial cancer-specific survival at 60 and 120 months was 92.3% and 83.1%, respectively. Although not significant, clinical/areolar disease had better survival (p=0.275). Anti-HER therapy (p=0.509) did not influence survival, with clinical stage being an isolated factor (p=<0.001). Comparing the clinical and pathological variables, the only one that was associated with performing the COM was age, in which patients aged 40-49 were associated with a higher rate of oncoplasty (p=0.002) and a risk ratio of 3.22 [CI 3.39-184.50]. COM did not influence local recurrence or survival (p=0.132) Conclusion: MPD is a rare condition, associated with multifocality/multicentricity, overexpression of HER 2 and difference between groups with clinical disease and another with subclinical/pathological disease. COM does not influence local recurrence or survival. More studies are needed to assess clinical/areolar disease, and the impact of advances in mastology on MPD.
Introduction: In recent decades, profound changes have occurred in the treatment of breast cancer, with evaluation of molecular subtypes, staging from a prognostic point of view, greater use of magnetic resonance imaging in the local extension of the disease, use of Targeted Therapies, in addition to the consolidation of oncoplastic surgery. Paget's Disease of the Breast (MPD), in this context, is a rare entity, being presented with limited case series. Initially described as a clinical entity, however, this fact is not unanimous, constituting a multifocal/multicentric disease. Objective: To assess the impact of changes in mastology in relation to MPD and the feasibility of performing oncoplastic breast surgery (OBS) in this disease. Methods: Retrospective study of patients seen at a Tertiary Oncology Hospital. In the presence of areolar alterations, MPD was characterized as clinical and in the others as subclinical/pathological, all with Paget's anatomopathological diagnosis. Clinical and prognostic data were evaluated, comparing the different clinical forms. Situations related to the performance of surgeries and their impact on local recurrence and survival were described. Descriptive statistics, comparison between groups (chisquare and Mann-Witney test) and survival (log-rank; Kaplan & Meier) were used. Cancer-specific survival was evaluated. Results: 85 patients were evaluated. The clinical form was present in 58.8% of the patients. Regarding staging, 27.1% had stage zero disease, with 92.9% multifocal/ multicentric disease. Most patients (83.5%) had the molecular subtypes HER 2 and Luminal HER 2. Patients with clinical MPD had a higher rate of "in situ" disease (p=0.028), being submitted to conservative treatment more frequently (p <0.001). Of the 43 patients with HER 2 invasive disease, most underwent anti-HER therapy. Mean follow-up was 71.2 ± 43.3 months. The actuarial cancer-specific survival at 60 and 120 months was 92.3% and 83.1%, respectively. Although not significant, clinical/areolar disease had better survival (p=0.275). Anti-HER therapy (p=0.509) did not influence survival, with clinical stage being an isolated factor (p=<0.001). Comparing the clinical and pathological variables, the only one that was associated with performing the COM was age, in which patients aged 40-49 were associated with a higher rate of oncoplasty (p=0.002) and a risk ratio of 3.22 [CI 3.39-184.50]. COM did not influence local recurrence or survival (p=0.132) Conclusion: MPD is a rare condition, associated with multifocality/multicentricity, overexpression of HER 2 and difference between groups with clinical disease and another with subclinical/pathological disease. COM does not influence local recurrence or survival. More studies are needed to assess clinical/areolar disease, and the impact of advances in mastology on MPD.
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Câncer de mama, Doença de Paget Mamária, Cirurgia conservadora da mama, Cirurgia oncoplástica, Breast cancer, Conservative breast surgery, Oncoplastic surgery, Paget's Disease Mammary