Brendan Delaney, MBA, Chief Operating Officer of Aadi Bioscience, provides an overview of malignant perivascular epithelioid cell tumor (PEComa) and its treatment.
As Mr. Delaney explains, PEComa is an ultra-rare family of sarcomas. Most PEComa begin as benign tumors which can be removed surgically. However, when these tumors are malignant, they become aggressive. Often it is diagnosed as a painful mass in the abdominal region. When it is possible, surgery is usually the preferred treatment for PEComa; however, due to the aggressive nature of these tumors when malignant, chemotherapy has historically been used. Unfortunately, chemotherapy has been shown in the literature to have minimal benefit for patients with PEComa.
Recently, Aadi Bioscience received FDA approval for albumin-bound sirolimus (Fyarro) for the treatment of adult patients with locally advanced unresectable or metastatic malignant PEComa.
To learn more about PEComa and other rare cancers, visit checkrare.com/diseases/cancers/