Tenosynovial Giant Cell Tumor, Diffuse Type
NCI Definition: A locally aggressive, diffusely infiltrating tumor, arising in the tendon sheath. It is composed of synovial-like mononuclear cells, hemosiderin-laden macrophages, foam cells, and inflammatory cells. Multinucleated osteoclast-like giant cells are usually present, although in a minority of cases they may be absent or rare. It predominantly affects young adults. Symptoms include joint swelling, pain, and joint effusion. 
Tenosynovial giant cell tumor, diffuse types most frequently harbor alterations in NOTCH1, TP53, PDGFRA, NOTCH3, and MECOM .
NOTCH1 Mutation, NOTCH1 Missense, TP53 c.217-c.1178 Missense, TP53 R175H, and TP53 Mutation are the most common alterations in tenosynovial giant cell tumor, diffuse type .
There is 1 clinical trial for tenosynovial giant cell tumor, diffuse type, of which 1 is open and 0 are completed or closed. Of the trial that contains tenosynovial giant cell tumor, diffuse type as an inclusion criterion, 1 is phase 2 (1 open).
Pd-0360324 and avelumab are the most common interventions in tenosynovial giant cell tumor, diffuse type clinical trials.
2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.