Abstract

Primary uterine angiosarcoma is exceedingly rare and has a poor prognosis. Moreover, the radiologic findings of this disease have not been previously documented. We present a case of a 62-year-old woman with primary uterine angiosarcoma who underwent abdominal hysterectomy and bilateral salpingo-oophorectomy. Histologically, interlacing vascular spaces were lined by endothelial cells showing nuclear pleomorphism and mitotic activity. Immunohistochemical staining was positive for the endothelial cell markers CD31, CD34, and Factor VIII, supporting the diagnosis of primary uterine angiosarcoma. Magnetic resonance imaging (MRI) revealed a heterogeneous mass with high and low signal intensity (T2 weighted) in the uterus and an intense contrast-enhanced anterior area within the mass (gadolinium enhanced, T1 weighted). The lesion was also enhanced on computed tomography (CT). Radiologically, the most helpful sign in the characterization of uterine angiosarcoma is marked heterogeneity on T2-weighted MRI with focal areas of high signal intensity, known as the “cauliflower-like appearance.” In addition, findings of a strongly enhanced lesion on gadolinium-enhanced T1-weighted MRI and contrast-enhanced CT also support the diagnosis of angiosarcoma