What's New in Uterine Sarcoma Research and Treatment?
Molecular pathology of uterine sarcoma
Recent research has improved our understanding of how changes in certain molecules can cause normal cells to become cancerous. For several years we have known that mutations (damage or defects) to DNA can alter important genes that regulate cell growth. If these genes are damaged, excess growth may result in cancer formation. Analysis of DNA from uterine sarcomas has revealed several changes in the genes that control cell growth.
Each human cell contains 23 pairs of chromosomes. Many endometrial stromal sarcomas (ESSs) have abnormalities in chromosomes 6, 7, or 17. Often, there is an abnormal “swapping” of DNA between chromosomes 7 and 17. Part of chromosome 7 goes to 17 and part of 17 goes to 7. This is known as a translocation. The swapping of DNA between the chromosomes leads to the formation of a new gene, called JAZF1/JJAZ. This gene may help the cells to become malignant. Finding it can confirm the diagnosis of ESS. A different translocation, called YWHAE/FAM22 occurs in undifferentiated uterine sarcomas (high-grade stromal sarcomas). Cancers with the YWHAE/FAM22 translocation tend to grow and spread more aggressively than those with the JAZF1/JJAZ translocation.
Scientists expect that discoveries such as these will eventually lead to new strategies for detection, prevention, and treatment.
New drugs, as well as new ways to give standard drugs are being tested. One drug, trabectedin (Yondelis®), was recently approved to treat leiomyosarcomas in the United States. Another drug, temozolomide, which is approved to treat brain tumors, also seems to help women with uterine leiomyosarcomas. Adjuvant radiation and chemotherapy continue to be evaluated for treatment of uterine sarcomas. New compounds are also being evaluated for soft-tissue sarcomas and may help women with uterine sarcomas. Some of these compounds act differently from traditional chemotherapy drugs and are called targeted therapies.
Last Medical Review: May 12, 2014 Last Revised: February 15, 2016