ANGIOSARCOMA TREATMENT

Angiosarcomas are aggressive soft-tissue sarcomas that are associated with high rates of local and distant recurrence and poor outcomes.^[1,2] Close review of patient series and tumor characteristics are increasingly identifying new prognostic factors and therapeutic approaches,^[3] but the rarity of this tumor type makes it challenging to determine optimal treatment strategies.

In an interview with Medscape, Sandra P. D'Angelo, MD, a medical oncologist with the Melanoma and Sarcoma Service at Memorial Sloan-Kettering Cancer Center (MSKCC) in New York, discussed how recent and emerging data are improving our understanding of angiosarcoma and, ultimately, pointing us toward new approaches in the treatment of this disease.

Primary and Secondary Angiosarcomas

Medscape: Let's start with some background on the presentation of angiosarcomas. Where do they typically arise?

Dr. D'Angelo: Angiosarcomas are most often seen in the head and neck area, as well as in the breast parenchyma.^[1] Radiation-associated angiosarcomas are the next most common presentation, and these develop usually in breast cancer patients who have undergone breast-conserving therapy followed by radiation therapy. This is not something that happens very often -- fewer than 1% of all breast cancer patients who receive radiation therapy will develop radiation-associated angiosarcoma.^[4] But we do see these tumors arise in this population. Other, less common presentation sites include the extremities and various organs, such as liver, lung, and mediastinum.

Surgical Margins and Outcome

Medscape: What do we know about prognostic factors and how they affect outcomes?

Dr. D'Angelo: There are only about 150 cases diagnosed each year in the United States, so most of what we know about angiosarcomas comes from retrospective case series.^[3,5-7] Historically, a few poor prognostic factors have been identified. One is tumor size > 5 cm. This is in line with what we see in general with soft-tissue sarcomas, where tumor size is generally predictive of worse outcomes.

High tumor grade has also been identified as a poor prognostic factor, but work done by Dr. Cristina Antonescu at MSKCC, a pathologist who focuses on angiosarcoma research, has shown that all angiosarcomas are of high grade, with the exception of primary breast angiosarcomas.^[5]

Other poor prognostic factors include older age, the presence of metastatic disease at the time of diagnosis, and poor performance status.

When we looked at the data from MSKCC, we found that having gross positive margins after surgery led to worse outcomes, whereas the use of adjuvant chemotherapy led to improved outcomes.^[3]

As with all sarcomas, surgical resection with the goal of obtaining gross negative margins is the treatment of choice. However, positive surgical margins are quite common in patients with angiosarcoma because of the infiltrative nature of the disease.

In our analysis, we found that patients with gross positive surgical margins had worse outcomes, but the presence of microscopic positive margins did not predict worse outcomes on multivariate analysis.^[3]Obtaining gross negative margins is of the utmost importance in the management of these types of cancers.