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.

Primary pulmonary epithelioid angiosarcoma with thyroid tumor history: A case report and literature review

“…Angiosarcoma is a rare disease, and thus, there is currently no standard treatment plan, particularly not for PPA. Patients with pulmonary angiosarcoma have been treated with surgical resection ( 5 , 11 , 17 , 22 ), adjuvant radiotherapy ( 8 , 20 , 22 ), chemotherapy ( 20 , 26 ), immunotherapy ( 27 , 28 ) and combined therapy ( 10 , 16 , 20 ). It has been proposed that surgery was the most effective treatment, which should be considered as early as possible for resectable tumors ( 5 , 17 ).…”

“…Xu et al ( 28 ) reported that a patient with PPA benefited from anti-PD-L1 treatment (pembrolizumab 200 mg) with high PD-L1 expression (70%); 9 days after the first pembrolizumab infusion, a CT scan demonstrated a confirmed size reduction of certain lesions compared with original lesions. A previous study indicated that a case of stage IV PPA had an excellent response to immunotherapy (pembrolizumab 2 mg/kg every 21 days) after progression on first-line chemotherapy (paclitaxel) and had stable disease after 1 year of immunotherapy treatment, which the patient was able to tolerate well ( 27 ). It has been indicated that patients with low expression of aldehyde dehydrogenase (ALDH) in primary pulmonary angiosarcoma have a favorable prognosis, whereas patients with high expression of ALDH have poor prognosis ( 20 ).…”

Complete resection of a pulmonary artery sarcoma involving the pulmonary valve and right ventricle outflow tract: a case report

“…A recent case report described promising treatments for metastatic pulmonary angiosarcoma with pembrolizumab. 9 However, further research on immunotherapy should be undertaken to investigate its efficacy and safety in this disease.…”

MYC Amplification in Epithelioid Angiosarcoma of the Urinary Bladder and Prostate Following Prostate Radiotherapy: A Case Report with a Novel Molecular Alteration

“…1 It most commonly affects adults and elder patients 2 and frequently arises in cutaneous sites and often presents within soft tissue, visceral organs, bone, and retroperitoneum. 3,4 The pathogenesis of angiosarcoma remains unclear, although definite risk factors include chronic lymphoedema, history of radiation, environmental carcinogens (vinyl chloride, thorium dioxide and arsenic) and several genetic syndromes. 3,5 Epithelioid angiosarcoma is a rare variant of angiosarcoma, which is characterized by solid growth pattern, with large atypical epithelioid or polygonal cells with ovoid vesicular nuclei, prominent nucleoli and abundant cytoplasm.…”

“…3,4 The pathogenesis of angiosarcoma remains unclear, although definite risk factors include chronic lymphoedema, history of radiation, environmental carcinogens (vinyl chloride, thorium dioxide and arsenic) and several genetic syndromes. 3,5 Epithelioid angiosarcoma is a rare variant of angiosarcoma, which is characterized by solid growth pattern, with large atypical epithelioid or polygonal cells with ovoid vesicular nuclei, prominent nucleoli and abundant cytoplasm. The epithelioid feature conveys prognostic significance for angiosarcoma.…”