Overview

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

Synonyms
Giant Cell Neoplasm of Tendon Sheath, Fibrous Histiocytoma of Tendon Sheath, Giant Cell Tumor of Tendon Sheath, Tendon Sheath Giant Cell Tumor, Tenosynovial Giant Cell Neoplasm, Giant Cell Tumor of Tenosynovium, Giant Cell Neoplasm of Tenosynovium, Tendon Sheath Giant Cell Neoplasm, Giant Cell Tumor of the Tenosynovium, Giant Cell Neoplasm of the Tenosynovium
Parent(s)
Synovial Neoplasm
Children
Tenosynovial Giant Cell Tumor, Diffuse Type and Malignant Tenosynovial Giant Cell Tumor

References

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.