Advancing the Treatment of Alveolar Soft Part Sarcoma

In season 4, episode 5 of Targeted Talks, Alice P. Chen, MD, medical officer in the Target Therapeutics 1 Section of the Investigational Drug Branch of the Cancer Therapy Evaluation Program at the National Cancer Institute, discusses important studies that are investigating treatment options for alveolar soft part sarcoma (ASPS).

According to Chen, there is only one therapy specifically granted FDA approval to treat ASPS.

“Alveolar soft part sarcoma is an extremely rare tumor. It occurs in less than 1% of all sarcomas, which, in general, is not that common to start with. Alveolar soft part sarcoma is one that is seen primarily in young adults,” explain Chen.

Atezolizumab (Tecentriq) was approved in December of 2022 based on findings from an open-label, single-arm study (Study ML39345; NCT03141684) of 49 adult and pediatric patients with unresectable or metastatic ASPS.

The findings showed that at the 1200 mg. dose for adult and 15 mg for children, atezolizumab achieved an objective response rate of 24% (95 CI, 13%-39%) with a duration of response (DOR) of 6 month or more in 67% of patients. Moreover, 42% of patients had a DOR of 12 months or more. The most common adverse events shown with atezolizumab during the study included musculoskeletal pain (67%), fatigue (55%), rash (47%), cough (45%), as well as nausea, headache, and hypertension (43% each).

Today, atezolizumab is being investigated multiple studies in combination with other therapies. One study is evaluating atezolizumab with or without bevacizumab (Avastin) in adult and pediatric patients aged 2 years or older to further understand the efficacy, safety, and pharmacokinetics of the agent (NCT03141684). On another study (Axiom; NCT05333458), atezolizumab is being administered with or without selinexor (Xpovio) in adult aged 18 years or older, explain Chen.

_REFERENCES:

_{FDA grants approval to atezolizumab for alveolar soft part sarcoma. News release. FDA.gov. December 9, 2022. Accessed April 26, 2023. https://bit.ly/3LcwZh6}