Primary Uterine Angiosarcoma

Primary uterine angiosarcoma is extremely rare and has a poor prognosis. Angiosarcoma (malignant hemangioendothelioma or hemangiosarcoma) is defined as a tumor of the endothelial cells that lined the blood vessels. It represents less than 1% of all soft tissue sarcomas (1). Although angiosarcomas can arise in any region of the body, most arise in the skin or superficial soft tissue of the elderly (1,2). The most common locations for angiosarcomas include skin, soft tissues, breast, spleen, liver and bone (1). Although rare, angiosarcomas have been reported to originate in the uterus, cervix, fallopian tube, ovary, parametrium, broad ligament and vagina. In the uterus, the vascular channels tend to infiltrate the myometrium and the tumor, if poorly differentiated, can display areas of solid growth (2). Uterine angiosarcomas are very rare tumor, with only 20 cases valid cases or primary uterine angiosarcomas described. Angiosarcomas of the uterus may be classified into primary and secondary cases. Predisposing factors for secondary angiosarcoma include ionizing radiation and chronic lymphedema after radical mastectomy (StewartTreves syndrome) (2). Primary uterine angiosarcoma is a malignant tumor of the vascular endothelium that occurs predominately in peri and postmenopausal women with uterine bleeding and anemia. We present a case of a 66-year-old woman with uterine angiosarcoma who underwent abdominal hysterectomy and bilateral salpingo-oophorectomy, showing the importance of immunohistochemical staining in making the final diagnose. Histologically, the tumor has anastomosing vascular channels lined by highly atypical endothelial cells. Immunohistochemical staining was positive for endothelial markers CD31 and vimentin, supporting the diagnosis of uterine angiosarcoma. Primary uterine angiosarcoma tend to demonstrate a highly malignant behavior. The major prognostic factors seems to be the size of the tumor at diagnosis and the presence of extra pelvic disease. No consensus exist for best possible therapy given the few cases from which to draw conclusions. Key-words: angiosarcoma; hemangioendothelioma; hemangiosarcoma.