Primary angiosarcoma of the breast: a case report

Abstract

Primary angiosarcoma of the breast is extremely rare. Radiologic findings are often non specific and may appear completely normal in one-third of cases with primary angiosarcoma. The prognosis is usually poor because of the high rates of local recurrence and early development of metastasis. Surgical removal followed by adjuvant chemotherapy seems improve the prognosis.We report a case of a 33- year-old woman with a highly vascular mass in her right breast which is suggestive of malignancy at radiology. Initial core needle biopsy showed a benign hemangioma. The patient underwent a mastectomy. The tumor histology showed papillary formations and vascular structures lined by atypical cells with hyperchromatic nucleus and eosinophilic cytoplasm with solid areas. The tumor cells expressed CD34 and CD31 but were negative for cytokeratin. The diagnosis of angiosarcoma grade III was made. The patient is now receiving chemotherapy. She is still alive.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1530481200889780

Angiosarcoma primario de mama y carcinoma papilar de tiroides sincrónico: presentación de un caso

“…Los angiosarcomas de novo o primarios suponen menos del 0,05% de todos los tumores primarios malignos de la mama y muestran un predominio por la mama derecha, como en nuestro caso (1,4,5).…”

“…Bajo grado o bien diferenciado, en el que no existen áreas papilares, sólidas, necróticas ni hemorrágicas; se objetivan vasos formados por una o dos capas de células endoteliales, sin mitosis (1,11). El análisis inmunohistoquímico muestra positividad para los anticuerpos antifactor VIII, CD31, CD34 y VEGF-R (1,5,12).…”

“…Como diagnóstico diferencial se incluye el cistosarcoma filodes, sarcoma estromal, carcinoma metaplásico, carcinoma de célula escamosa, mioepitelioma, fibromatosis, fibrosarcoma, liposarcoma y hemangioma benigno (1,5).…”

“…El angiosarcoma mamario muestra un comportamiento sumamente agresivo, cuyo pronóstico se relaciona directamente con el grado histológico (1,5,8,13). La supervivencia a 5 años en tumores de bajo grado es del 91%, 68% en los de grado intermedio y 14% en los de alto grado (1,14).…”

Primary and Secondary Angiosarcoma of the Breast

“…On histopathological analysis the lesions are notable for irregular vascular formations evident on with hyperchromatic and irregular nuclei. The diagnosis can be clarified by immunohistologic staining for the endothelial marker CD31, the most sensitive and specific indicator of angiogenic proliferation; however, the lesions will also stain positive for the vascular markers Factor VIII, and Fli1, and will usually at least be weakly positive for CD34 (15)(16)(17) (Figure 2). …”