Although rare, chondrosarcomas — malignant cartilaginous neoplasms — are the second most common primary malignant tumors of bone. They have not been shown to respond to chemotherapy or radiation, leaving surgery as the only effective treatment option. Duke Health’s specialized team provides complex resection and reconstruction with innovative approaches including 3D printing implants to reconstruct vital bones and joints while also advancing treatments for chondrosarcoma.

Orthopaedic surgical oncologist Julia Visgauss, MD, Duke’s division chief of orthopaedic oncology, notes that often, the tumors present with large masses in the hips or pelvis, rendering surgery very morbid with intense recovery. And in the case of metastasis, the disease is incurable.

Visgauss is leading research to understand the mechanisms of aggressive behavior in chondrosarcoma, and the development of novel treatment options using Duke’s robust biobank of sarcoma tissues. As an advanced cancer center, Duke also provides patients access to the latest treatments through adjuvant therapy clinical trials.

Studies have shown promise for the gene LCP1 as a determinant of malignancy. “This gene and the protein it encodes are important for how cells move and invade through tissues,” Visgauss says. “We’ve developed tools that show when this gene is overexpressed, the cancer cell’s abilities to migrate and attach increase, and when we knock out the gene, those abilities all decrease.”

Rodent studies have validated this finding. “Mice injected with chondrosarcoma cells that highly express LCP1 develop numerous pulmonary tumors. However, when we knock out this gene, virtually no tumors develop,” Visgauss says. “We are currently studying some drugs that target these mechanisms with very promising preclinical results.”

Visgauss’s team is also studying the role that the tumor microenvironment plays in the recruitment of normal cells that may contribute to tumor cell aggressiveness and immune evasion through cell-cell signaling. “For many years, it was thought that chondrosarcoma was an immune desert, but our studies have shown that there are immune cells. Existing immunotherapies don’t work in chondrosarcoma because the mechanisms are completely different than other cancers,” says Visgauss. “These studies are opening exciting avenues for novel therapeutics to prevent metastatic disease.”

Due to their rarity, it’s common for sarcomas to go undiagnosed for a long time. If a patient has pain that doesn’t resolve or an abnormal component to the pain and a tumor is suspected, cross-sectional imaging can help to reach a diagnosis sooner. “Early referral can make the process more efficient and optimized,” says Visgauss. “For example, exactly how and where a biopsy is performed can affect later resection and treatment. If we can plan and do the biopsy here, we can make sure that the technique will best facilitate later resection and that a rapid and accurate diagnosis is made by our expert pathologists.”

Visgauss notes that while chondrosarcoma has primarily been considered a condition of older patients, patients in their 30s to 50s are increasingly diagnosed. “We’re always willing to review imaging and answer questions to help providers decide whether to refer so no one is missed,” she says.