Introduction: Kasabach-Merritt syndrome is characterized by the association of a consumptive thrombohemorrhagic disorder and angioma occurring usually in children. In the present study, a case of Kasabach-Merritt syndrome associated to an angiosarcoma of the breast is reported.
Clinical history: The tumor together with the thrombohemorrhagic disorder manifested in a 28-year-old woman. The patient underwent mastectomy. The tumor recurred in the same site 2 years later. The patient died of severe anemia 8 years after the first appearance of the angiosarcoma.
Material and methods: Tissues were formalin fixed and paraffin embedded; in addition, selected sections were immunohistochemically stained.
Results and conclusions: A well-differentiated angiosarcoma was visible throughout the removed organ. A review of the literature showed that only 6 cases of Kasabach-Merritt syndrome associated to malignant vascular tumors have been previously reported. All these 6 cases occurred in adult patients. On the contrary, Kasabach-Merritt syndrome associated to benign vascular tumors affects children. This is the first case occurring in the breast.