Role of Immunotherapy in Pulmonary Angiosarcoma: A Case Report

Abstract

Pulmonary angiosarcoma is a rare clinical entity with a poor prognosis and no established therapeutic strategies. We present the first case to our knowledge of metastatic pulmonary angiosarcoma, treated with checkpoint inhibitor immunotherapy, and have an excellent response. Until now, patient has been treated with immunotherapy for 1 year, and his disease is stable and well-tolerated.

Keywords: Immunotherapy; Prembrolizumab; Pulmonary angiosarcoma.

Fig. 1

Representative pathologic sections of pulmonary angiosarcoma. H and E stain and immunohistochemistry revealed that the tumor cells were positive for CD31, ERG, and Vimentin.

Fig. 2

Imaging results from initial hospitalization. a Chest CT shows a left hilum lung mass with enlargement of mediastinal and right hilum lymph nodes. b Bone scan shows an increased uptake in left second rib with high suspicion of metastasis. CT, computed tomography.

Fig. 3

Imaging results before and after the treatment of pembrolizumab. a Contrast-enhanced axial CT (after 3 cycles of paclitaxel) demonstrates complete atelectasis of the left lung due to a large left perihilar mass. b Image after 3 cycles of pembrolizumab show decreasing in size of left lung tumor to 34 × 37 mm (compare with 88 mm before). CT, computed tomography.