Michael J. Wagner, MD, assistant professor, University of Washington School of Medicine and Fred Hutchinson Cancer Research Center, explains how immunotherapy may be a promising treatment approach for patients with angiosarcoma.

There is a significant unmet medical need for new treatments in angiosarcoma, according to Wagner. There is no established standard of care for the disease, and efficacy of existing options is not as strong as desired, Wagner says.

Findings from small studies and case reports suggest that immunotherapy could address that unmet need, Wagner adds. For example, Wagner led the phase 2 DART trial (NCT02834013) evaluating ipilimumab (Yervoy) in combination with nivolumab (Opdivo) for patients with metastatic or unresectable angiosarcoma. 

One-quarter of patients in the trial responded to treatment, according to Wagner, who expresses excitement for what these findings could mean for the field going forward.

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Michael J. Wagner, MD, explains how immunotherapy may be a promising treatment approach for patients with angiosarcoma.

Dr Wagner or the Potential Value of Immunotherapy in Angiosarcoma

Michael J. Wagner, MD, assistant professor, University of Washington School of Medicine and Fred Hutchinson Cancer Research Center, discusses molecular pathways that are becoming relevant in angiosarcoma.

Recent breakthroughs in genetic sequencing have led to the identification of  molecular pathways that drive angiosarcoma, according to Wagner.

Investigators are exploring treatments targeting the MAPK pathway, as well as PIK3A, and mTOR, Wagner adds. Developing new agents can be challenging in angiosarcoma, but this knowledge may allow physicians to explore off-label regimens, Wagner concludes.

Michael J. Wagner, MD, discusses molecular pathways that are becoming relevant in angiosarcoma.

Dr Wagner on Molecular Pathways in Angiosarcoma

Michael J. Wagner, MD, assistant professor, University of Washington School of Medicine and Fred Hutchinson Cancer Research Center, discusses known risk factors for developing angiosarcoma.

Radiation exposure is a known cause of angiosarcoma, particuarly patients who have had breast-conserving therapy for breast cancer, according to Wagner. However, only a small fraction of those who are exposed to radiation in this way will eventually develop angiosarcoma, Wagner adds.

It has recently been discovered that exposure to ultraviolet light is associated with angiosarcoma, particularly when the disease presents on the face or scalp, Wagner says. Investigators have already started incorporating that information into their clinical trials, Wagner concludes. 

Michael J. Wagner, MD, discusses known risk factors for developing angiosarcoma.

Dr. Wagner on Risk Factors for Angiosarcoma

Michael J. Wagner, MD, assistant professor, University of Washington School of Medicine and Fred Hutchinson Cancer Research Center, discusses the challenge of diagnosing angiosarcoma.

Even as a specialist working in a referral center, Wagner only sees 15 to 20 patients with angiosarcoma in a given year. Diagnosing angiosarcoma is challenging because it can appear in almost every system in the body and the disease often looks like many other conditions.

Wagner says angiosarcoma can present as a bruise that does not go away or a purple lesion on the skin or scalp. Some patients experience pain at the site of the disease or, if angiosarcoma arises in the lungs, they may experience shortness of breath or cough.

Michael J. Wagner, MD, discusses the challenge of diagnosing angiosarcoma.

Dr. Wagner on Diagnosing Angiosarcoma

Michael J. Wagner, MD, assistant professor, University of Washington School of Medicine and Fred Hutchinson Cancer Research Center, discusses the importance of cooperative research groups in angiocarcinoma.

Angiosarcoma is a rare subset of an already rare disease, soft tissue sarcomas. Due to the lack of patients, it can be difficult for single institutions to recruit enough participants to conduct clinical trials.

Clinical trials requires collaboration, according to Wagner, who calls cooperative groups like EGOC, the Alliance, and SWOG "critical" for conducting research. Findings from the Angiosarcoma Project discovered the connection between exposure to ultraviolet light and angiosarcoma.

Michael J. Wagner, MD, discusses the importance of cooperative research groups in angiocarcinoma.

Angiosarcoma | Videos

Dr Wagner or the Potential Value of Immunotherapy in Angiosarcoma

Dr Wagner on Molecular Pathways in Angiosarcoma

Dr. Wagner on Risk Factors for Angiosarcoma

Dr. Wagner on Diagnosing Angiosarcoma

Dr. Wagner on the Value of Cooperative Research Groups in Angiosarcoma