Overview

NCI Definition: A malignant neoplasm characterized by the chromosomal translocation t(X;18)(p11;q11). It can occur at any age, but mainly affects young adults, more commonly males. Although any site can be affected, the vast majority of the cases arise in the deep soft tissues of extremities, especially around the knee. Microscopically, synovial sarcoma is classified as monophasic (with a spindle or epithelial cell component) or biphasic (with both spindle and epithelial cell components). Synovial sarcomas can recur or metastasize to the lungs, bones, and lymph nodes. [1]

Synovial sarcomas most frequently harbor alterations in ARID1B, TGFBR2, NF2, CTNNB1, and ATM [2].

Most Commonly Altered Genes in Synovial Sarcoma

TGFBR2 Loss, ATM Mutation (germline), ATM Mutation, ABL1 Mutation, and WRN Mutation are the most common alterations in synovial sarcoma [2].

Top Alterations in Synovial Sarcoma

Significant Genes in Synovial Sarcoma

SMARCA4 +

SMARCB1 +

SS18 +

SSX1 +

SSX2 +

SSX4 +

Disease Details

Synonyms
Synovial Sarcoma, Not Otherwise Specified, SARCOMA, SYNOVIAL, MALIGNANT, SS, Synovial Sarcoma, NOS
Parent(s)
Malignant Synovial Neoplasm
Children
Mediastinal Synovial Sarcoma, Biphasic Synovial Sarcoma, Monophasic Synovial Sarcoma, and Spindle Cell Synovial Sarcoma
OncoTree Name
Synovial Sarcoma
OncoTree Code
SYNS

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 4. 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.