Bilateral ovarian hemangiomas associated with diffuse hemangioendotheliomatosis: a case report

A patient who had disseminated vascular tumors involving the bilateral ovaries, bilateral lungs and pleura, pericardium, and mediastinum is reported. The tumors were histologically of the capillary, and partly the cavernous, type of hemangioma. However, endothelial cell growth was prominent in some areas, especially in the lung, and the histology of the lung tumor resembled epithelioid hemangioendothelioma or intravascular bronchiolo-alveolar tumor (IVBAT). In the endocardium of the right atrium, an endothelial tumorous projection was observed, and there were tiny foci of tumor cell nests in the abdominal venous wall. Small lymphangiomas were also found in the subcapsular region of the spleen. These findings suggest that there had been an abnormal proliferation of systemic endothelial cells and that tumors of endothelial cell origin with diverse histological patterns developed with this condition as a background. The autopsy finding of fibrin thrombi in multiple organs as well as laboratory data including thrombocytopenia suggest that this case belongs to the "Kasabach-Merritt syndrome."