Dr Randall on the Complexity of PEComas

“We want to make sure that it’s really a complete team, that they’re coming together, hopefully at least weekly, to communicate about the specific nuanced decision-making around any given patient with PEComa.”

R. Lor Randall, MD, FACS, the David Linn Endowed Chair for Orthopedic Surgery, chair of the Department of Orthopedic Surgery, and professor in the Department of Orthopedic Surgery at the University of California Davis Health, emphasized the complex natureof perivascular epithelioid cell tumors (PEComas) and the importance of diagnostic accuracy and multidisciplinary care in managing this rare disease.

Although PEComa represents a small fraction of sarcomas, its biologic heterogeneity requires a deliberate and coordinated approach to ensure optimal outcomes. Randall explained that even within sarcoma centers, misclassification remains a risk if tumor specimens are not reviewed by pathologists with dedicated expertise in sarcoma pathology. Immunohistochemistry, molecular assays, and translocation testing are essential to confirm the diagnosis, distinguish PEComa from other sarcomas, and guide appropriate therapy, according to Randall. He stressed that without this rigorous diagnostic process, there is potential for inappropriate treatment decisions, which can significantly affect patient outcomes.

Beyond pathology, Randall underscored the necessity of a comprehensive, multispectral treatment plan guided by a fully integrated care team. This approach requires medical oncologists who have current knowledge of emerging biologically targeted therapies, as well as radiation oncologists and surgical oncologists capable of weighing the risks and benefits of local control strategies, Randall noted. He highlighted the value of weekly multidisciplinary tumor boards, where all relevant specialists collaborate to reach consensus on individualized management plans. These forums provide the opportunity to integrate pathology results, imaging, systemic therapy considerations, and patient-specific factors into a cohesive strategy, he stated.

Importantly, Randall cautioned against defaulting to immediate surgical resection or radiotherapy without a multidisciplinary review. Instead, he advocated for deliberative decision-making that incorporates molecular and clinical insights to determine the best sequencing of therapies. As biologically targeted options continue to expand for sarcoma subtypes, their potential role in PEComa management should be carefully assessed within the context of each patient’s disease course, he reported.

Randall concluded that managing PEComa requires a high level of diagnostic precision and a collaborative framework that integrates pathology, systemic therapy, and local treatment modalities. By committing to accurate classification and consistent multidisciplinary communication, oncologists can better navigate the biologic variability of PEComa and deliver patient-centered, evidence-informed care.