NCI Definition: A tumor usually arising in the synovium of joints, bursa or tendon sheath. It is characterized by the presence of mononuclear cells, multinucleated osteoclast-like giant cells, hemosiderin-laden macrophages, foam cells, and an inflammatory infiltrate. According to the growth pattern, it is classified as localized or diffuse. [1]

Tenosynovial giant cell tumors most frequently harbor alterations in NOTCH1, TP53, PDGFRA, NOTCH3, and MECOM [2].

Most Commonly Altered Genes in Tenosynovial Giant Cell Tumor

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 [2].

Top Alterations in Tenosynovial Giant Cell Tumor

Disease Details

Giant Cell Neoplasm of the Tenosynovium, Giant Cell Tumor of Tenosynovium, Tendon Sheath Giant Cell Neoplasm, Giant Cell Neoplasm of Tenosynovium, Giant Cell Tumor of the Tenosynovium, Tenosynovial Giant Cell Neoplasm, Giant Cell Neoplasm of Tendon Sheath, Tendon Sheath Giant Cell Tumor, Fibrous Histiocytoma of Tendon Sheath, Giant Cell Tumor of Tendon Sheath
Synovial Neoplasm
Tenosynovial Giant Cell Tumor, Diffuse Type and Malignant Tenosynovial Giant Cell Tumor


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

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.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.