Pulmonary epithelioid hemangioendothelioma. case report

Abstract

In 1983 an uncommon type of lung tumor was reported in 20 patients, it was then named IVBAT (intravascular, bronchioal- veolar tumor). The patients presented with multiple slow gro- wing pulmonary nodules, that ranged microscopically from (apparently) benign looking granulomas to clearly developed sarcomas. Eventually the tumor was found to be of vascular origin, and was called epithelioid hemangio endothelioma (EHE), later on it was also reported in other anatomical regions. We describe here, as far as we could find, the first costarican case of this particular neoplasia. A 42 year old male was referred to us with disseminated metastatic disease. He had been seen 16 years prior with asymptomatic, bilateral, small lung nodules. At that time he underwent a lung biopsy and the diagnosis of epitheliod hemangioendothelioma was made. He had remained stable, on no therapy, until his current and final presentation. We elected to biopsy his lung again to verify the histology and to see weather the tumor had under gone dif ferentiation. He did not respond to chemotherapy and died shortly thereafter. The original lung biopsy material was compared to the recent one, finding much more cellularity and atypiae in the latter one. Immuno-histochemistry studies were carried out on the tissue samples and the endothelial origin of the tumor was demonstrated. EHE is an unusual type of neoplasia, it grows very slowly and has low metastatic potential in spite of its vascular origin, interestingly some authors have shown a hormonal dependency and this needs to be explored to see if therapeutic applications can be developed.