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Angiossarcoma primário da mama : caso clínico
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Resumo(s)
O angiossarcoma da mama é uma neoplasia rara, que representa 0,05% das neoplasias malignas da mama, contudo corresponde ao tipo de sarcoma mamário mais frequente. É uma neoplasia vascular maligna que surge em pele ou tecido mamário exposto à radioterapia (angiossarcoma associado à radioterapia) ou, menos frequentemente, em tecido não irradiado (angiossarcoma primário).
Relativamente ao angiossarcoma primário da mama, não se reconhecem fatores de risco e a etiologia molecular ainda se encontra em investigação. É uma neoplasia que ocorre predominantemente no tecido mamário e, contrariamente ao angiossarcoma associado à radioterapia, o envolvimento cutâneo é raro. Habitualmente ocorre em mulheres com idade média de 30-40 anos. Clinicamente manifesta-se por um nódulo mamário de crescimento rápido ou por assimetria mamária. Imagiologicamente, os achados são inespecíficos, sendo a RMN o método mais sensível e específico. A biopsia mamária é a abordagem diagnóstica inicial preferível, devendo ser complementada com excisão total do tumor. Microscopicamente é uma neoplasia vascular infiltrativa, constituída por canais vasculares anastomosados com atipia endotelial. É essencial o diagnóstico diferencial com outras neoplasias benignas e malignas, associadas a terapêuticas e prognósticos muito distintos.
Neste trabalho descreve-se um caso clínico de angiossarcoma primário da mama do Serviço de Anatomia Patológica do Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, e realiza-se ainda revisão bibliográfica das características clínicas, diagnósticas, terapêuticas e prognósticas deste tipo de neoplasia.
Esta neoplasia reveste-se de desafios importantes no seu diagnóstico, e possui um fenótipo biologicamente agressivo, associado a um elevado potencial de metastização, e mau prognóstico. A avaliação anatomopatológica constitui um dos pilares no diagnóstico desta entidade. Não obstante, é fundamental a integração célere e multidisciplinar desta, com as características clínicas e imagiológicas de forma a adequar a terapêutica a implementar, considerando as características individuais de cada doente, e o estadiamento clínico-patológico do tumor.
Breast angiosarcoma is a rare neoplasm, accounting for 0,05% of malignant breast neoplasms, but it represents the most common type of mammary sarcoma. It is a malignant vascular neoplasm that arises in the skin or breast tissue exposed to radiotherapy (radiation-associated angiosarcoma) or, less commonly, in non-irradiated tissue (primary angiosarcoma). Regarding primary breast angiosarcoma, no recognized risk factors are identified, and the molecular etiology is still under investigation. It is a neoplasm that predominantly occurs in breast tissue, and, unlike radiation-associated angiosarcoma, cutaneous involvement is rare. It typically occurs in women with an average age of 30-40 years. Clinically, it presents as a rapidly growing breast lump or breast asymmetry. Imaging findings are nonspecific, with MRI being the most sensitive and specific method. Breast biopsy is the preferred initial diagnostic approach, complemented by total tumor excision. Microscopically, it is an infiltrative vascular neoplasm composed of anastomosed vascular channels with endothelial atypia. Differential diagnosis with other benign and malignant neoplastic entities is essential, because are associated with different therapies and prognoses. This paper describes a clinical case of primary breast angiosarcoma from the Pathological Anatomy Service of the Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, and includes a literature review of clinical, diagnostic, therapeutic, and prognostic characteristics of this type of neoplasm. This neoplasm poses significant challenges in its diagnosis, exhibiting a biologically aggressive phenotype associated with a high potential for metastasis and poor prognosis. Anatomopathological evaluation is a cornerstone in diagnosing this entity. Nevertheless, it is essential to promptly and multidisciplinarily integrate it with clinical and imaging characteristics to tailor the therapy to be implemented, considering the individual characteristics of each patient and the clinical-pathological staging of the tumor.
Breast angiosarcoma is a rare neoplasm, accounting for 0,05% of malignant breast neoplasms, but it represents the most common type of mammary sarcoma. It is a malignant vascular neoplasm that arises in the skin or breast tissue exposed to radiotherapy (radiation-associated angiosarcoma) or, less commonly, in non-irradiated tissue (primary angiosarcoma). Regarding primary breast angiosarcoma, no recognized risk factors are identified, and the molecular etiology is still under investigation. It is a neoplasm that predominantly occurs in breast tissue, and, unlike radiation-associated angiosarcoma, cutaneous involvement is rare. It typically occurs in women with an average age of 30-40 years. Clinically, it presents as a rapidly growing breast lump or breast asymmetry. Imaging findings are nonspecific, with MRI being the most sensitive and specific method. Breast biopsy is the preferred initial diagnostic approach, complemented by total tumor excision. Microscopically, it is an infiltrative vascular neoplasm composed of anastomosed vascular channels with endothelial atypia. Differential diagnosis with other benign and malignant neoplastic entities is essential, because are associated with different therapies and prognoses. This paper describes a clinical case of primary breast angiosarcoma from the Pathological Anatomy Service of the Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, and includes a literature review of clinical, diagnostic, therapeutic, and prognostic characteristics of this type of neoplasm. This neoplasm poses significant challenges in its diagnosis, exhibiting a biologically aggressive phenotype associated with a high potential for metastasis and poor prognosis. Anatomopathological evaluation is a cornerstone in diagnosing this entity. Nevertheless, it is essential to promptly and multidisciplinarily integrate it with clinical and imaging characteristics to tailor the therapy to be implemented, considering the individual characteristics of each patient and the clinical-pathological staging of the tumor.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2024
Palavras-chave
Neoplasias da mama Angiossarcoma Angiossarcoma primário da mama Diagnóstico diferencial Histopatologia Imunohistoquímica