Dr Riedel on the Role of the Multidisciplinary Team in PEComa Management

“At various levels, having a knowledge base about the disease state and then putting that in the context of a team is an effort to improve patient outcomes.”

Richard F. Riedel, MD, a professor of medicine in the Department of Medicine at the Duke University School of Medicine, as well as a member of the Duke Cancer Institute, discussed the importance of multidisciplinary perivascular epithelioid cell tumor (PEComa) management, as well as the importance of facilitating collaboration between community and academic oncologists when diagnosing and treating patients with this disease.

A multidisciplinary approach is essential to optimizing clinical outcomes in patients, particularly in the context of ultra-rare malignancies, such as PEComa, Riedel began. Involving subspecialized pathologists with sarcoma expertise facilitates accurate and timely histopathologic diagnosis, he said. Similarly, surgical management by oncologic surgeons experienced in treating patients with sarcoma ensures that resections are performed with curative intent when feasible, particularly in cases of localized disease, he emphasized. In addition, medical oncologists with specific expertise in rare sarcomas are more likely to implement evidence-based systemic therapies, including agents approved for PEComa and the appropriate application of mTOR inhibitors, he stated.

Often, oncologists in community-based settings are unaware of the availability of nab-sirolimus (Fyarro), the agent approved for the management of PEComa, Riedel noted. Therefore, disease-specific expertise is vital at multiple levels to guide the clinical decision-making process within a collaborative, team-based framework, he reported. These efforts collectively contribute to improved outcomes, even in the management of ultra-rare neoplasms, he continued.

Recent advances in PEComa management are primarily attributable to enhanced understanding of the molecular and pathophysiologic underpinnings of the disease, which have enabled the development and application of rational targeted therapies, according to Riedel. Given the rarity of PEComa, it is imperative that patients with suspected disease are evaluated at specialized centers—typically academic institutions with dedicated sarcoma programs—where multidisciplinary teams can provide comprehensive diagnostic and therapeutic care, he highlighted. Collaboration between academic centers and community practitioners remains fundamental to optimizing patient care, Riedel concluded.