By Peter Ciszewski;  September 27, 2019.

The National Comprehensive Cancer Network (NCCN) updated its Clinical Practice Guidelines in Oncology to include a category 1 recommendation for pexidartinib (Turalio) for the treatment of patients with tenosynovial giant cell tumor (TGCT).

In August, the U.S. Food and Drug Administration (FDA) granted approval to pexidartinib for the treatment of adult patients with symptomatic TGCT associated with severe morbidity or functional limitations and not responsive to improvement with surgery.

TGCT is a rare tumor that affects the synovium (thin layer of tissue that covers the surfaces of the joint spaces) and tendon sheaths (layer of membrane that covers tendons, which are fibrous tissue that connect muscle to bone). The tumor is rarely malignant but causes the synovium and tendon sheaths to thicken and overgrow, causing damage to surrounding tissue. 

The NCCN updated its guidelines with this category 1 recommendation for pexidartinib based on the randomized, placebo-controlled Phase 3 Study (ENLIVEN) for patients with TGCT, led by William D. Tap, MD, Chief of the Sarcoma Medical Oncology Service, and published in The Lancet in June of this year.

A category 1 recommendation means that based upon high level of evidence, there is uniform consensus that the intervention is appropriate. The updated NCCN guidelines outline the latest recommendations, helping to inform therapeutic decisions for patients.

 To learn more about TGCT, visit our TGCT Learning Center.