Clinical activity of checkpoint inhibitors in angiosarcoma: A retrospective cohort study

Abstract

Background Systemic treatments for angiosarcoma remains an area of unmet clinical need. The authors conducted this retrospective study to assess the clinical activity of checkpoint inhibitors in patients with angiosarcoma. The primary objective was to assess the objective response rate, and the secondary objective was to assess the progression‐free and overall survival durations and disease control rate. Methods Patient data were obtained using The University of Texas MD Anderson Cancer Center Tumor Registry database. The final study population was refined to only include patients who had undergone pembrolizumab monotherapy. The objective response rate was evaluated using RECIST/irRECIST version 1.1. Progression‐free survival and overall survival were defined as the time from the initiation of immunotherapy to disease progression or recurrence, death, or last follow‐up and to death or last follow‐up, respectively. Results The final cohort comprised 25 patients. Most patients had metastatic disease (72%) and had undergone at least two lines of systemic therapy (80%) before starting pembrolizumab. The objective response rate was 18%, whereas the disease control rate was 59%. The median progression‐free survival duration was 6.2 months and was not significantly different between the cutaneous (4.7 months) and visceral angiosarcoma (6.2 months) groups (p = .42). The median overall survival duration was 72.6 months. Toxicities were recorded for eight patients, with fatigue, anemia, constipation, and rash being the most common. Conclusions Pembrolizumab shows durable clinical activity in angiosarcoma. These findings suggest that checkpoint inhibition as monotherapy or combination therapy is likely to have a high probability of success.© 2022 American Cancer Society. Lay summary This is the largest retrospective study to assess the clinical activity of checkpoint inhibitor monotherapy in angiosarcomas. The study includes an adequate number of patients with visceral angiosarcoma that enabled to obtain meaningful clinical insights that were previously unavailable. Our findings indicate an improvement in progression‐free survival with pembrolizumab that is comparable to other active agents in angiosarcoma. Pembrolizumab monotherapy in angiosarcomas also has a favorable tolerability profile. Our findings emphasize the need for prospective studies to evaluate the activity of pembrolizumab monotherapy and combination therapy.

A bleeding heart: case report and review of pericardial angiosarcoma

“…Pembrolizumab showed a durable clinical response with mPFS of 6.2 months and OS of 72.6 months. 27 Future prospective studies evaluating targeted therapies and immunotherapy in cardiac angiosarcoma would be valuable, even though the rarity of the disease limits the scope of conducting large-scale studies.…”

Radiotherapy combined with anti-PD-1 and TKI for primary cardiac angiosarcoma considering the joint assessment of TLSs and PD-L1: a case report

“…Furthermore, targeted anti-angiogenic drugs and immune checkpoint inhibitors(ICIs) have been applied in clinical practice and achieved good results [ 10 – 12 ].Especially in angiosarcoma, data on the efficacy of immunotherapy are few but consistent in demonstrating excellent antitumour activity. A retrospective study found that patients with visceral angiosarcoma, including cardiac angiosarcoma, treated with pembrolizumab as monotherapy can also obtain progression-free survival (PFS) similar to other systemic treatments [ 13 ].…”

Tumor Immune Microenvironment and Its Clinicopathological and Prognostic Associations in Canine Splenic Hemangiosarcoma

“…In view of an approach to increasingly personalized medicine, the characterization of the immune landscape of a tumor becomes an important factor in the identification and stratification of patients who are likely to benefit from immunotherapy-based treatment strategies. This is particularly important for HSA which, even in human medicine, has limited therapeutic possibilities and a poor prognosis and in which emerging data suggest that alternative approaches with the use of immune checkpoint inhibitors are effective [86]. Con-tradicting results emerged from a retrospective study comparing the clinical characteristics and the results of targeted exome sequencing, transcriptome sequencing, and immunohistochemistry analyses in human patients with histologically confirmed angiosarcoma treated with immune checkpoint blockade-base therapy, where it was demonstrated that neither PD-L1 expression nor presence of TILs at baseline appears necessary for a response to immune checkpoint blockade (ICB) therapy [86].…”