Sydney Stern, Director of Giant Cell Tumor Programs at the Life Raft Group, Ph.D. Candidate at the University of Maryland Baltimore, and tenosynovial giant cell tumor (TGCT) patient, gives an overview of TGCT.

As Ms. Stern explains, TGCT are a group of rare, benign tumors that involve the synovium, bursae and tendon sheath. While these tumors are benign, they can grow and cause extreme damage to the surrounding tissues. TGCT is characterized by over-expression of colony-stimulating factor 1 (CSF-1) as well as recruitment and accumulation of cells that express CSF-1 receptor (CSF-1R) in the synovium.

 

 

There are generally two types of TGCT, a nodular and a more diffuse type. As Ms. Stern explains, the nodular form of TGCT can typically be treated with synovectomies, while the more diffuse form will usually require medical therapy. For the latter diffuse type of TGCT, the FDA-approved CSF1R inhibitor, pexidartinib, can be effective. Other investigational drugs like DDC-3014 and AMB-05X are currently being evaluated for the treatment of TGCT.

For more TGCT resources, visit www.TGCTsupport.org

To learn more about this rare disease, visit our TGCT Learning Center here.