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No standard therapy for pulmonary epithelioid hemangioendothelioma (PEH) has yet been established due to the rarity of the disease, the lack of clear standards for treatment and the partial-to-complete spontaneous regression. This report describes three cases of PHE manifested as bilateral intrapulmonary masses with an initial diagnosis conducted by thoracoscopic lung biopsy. These patients demonstrated a partial response to combination chemotherapy with carboplatin, paclitaxel, bevacizumab or endostar, and an improvement in clinical status. Furthermore, we reviewed the literature regarding such patients who received chemotherapy and immunotherapy; this indicated that patients with PEH demonstrated a good partial response to chemotherapy with carboplatin, paclitaxel, bevacizumab, thalidomide and α-interferon. Overall, combination chemotherapy regimens may hold therapeutic potential for the treatment of this rare disease.

Treatment of pulmonary epithelioid hemangioendothelioma with combination chemotherapy: Report of three cases and review of the literature