A CASE OF A PULMONARY EPITHELIOID HEMANGIOENDOTHELIOMA TRANSFORMED INTO AN EPITHELIOID ANGIOSARCOMA

Epithelioid hemangioendothelioma (EHE) is an uncommon vascular tumor characterized by epithelioid or histiocytoid cells exhibiting primitive vascular differentiation. This lesion may involve lung, soft tissue, bone, liver, breast, brain andlymphnodes. The pulmonary EHE is of “borderline malignancy”, the prognosis is unpredictable (mean survival 4.6 years) and death usually occurs for a progressive respiratory failure. About 50 cases have been reported in literature. In February 2000 a 20-year-old woman with a one year history of dry cough had a HRCT scan showing multiple bilateral nodules in the lungs. The patient underwent thoracoscopic lung biopsy and an interstitial lung disease was suspected. After a nine months course of corticosteroid therapy a remission of symptoms was obtained and the HRCT scan periodically perfomed for three years did not show a progression of the disease. In September 2004 she was admitted to our Department for a suspected pneumonia and a bronchoscopy was performed. The histological examen of bronchial biopsies revealed a poorly differentiated neoplasm immunohistochemically positive for vimentin and endothelial markers (factor VIII-related antigen and CD34). A diagnosis of angiosarcoma (malignant hemangioendothelioma) was made. Chemotherapy with paclitaxel (80 mg/mq 1, 8, 15 every 28 days for three cycles) was admnistered but after six months from the diagnosis the patient died. The revision of the former CT scan and histological examen suggested a diagnosis of pulmonary EHE (intravascular bronchiolo-alveolar tumor- IVBAT). At our knowledge, this is the first reported case of EHE, a low grade neoplasm, transformed into a very high grade angiosarcoma.