North American Undifferentiated Embryonal Sarcoma of the Liver Registry

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare form of liver cancer accounting for about 9-15% of all liver cancers seen in children. Patients usually present with solitary liver tumors ranging from 10-30cm in size between the ages of 5-12 years. Metastatic spread of disease is rare.

Children with UESL are currently treated with a combination of sarcoma chemotherapy and surgical resection, including partial removal of the liver and liver transplantation. The current survival for children undergoing combination therapy is >90% at 5yrs; however, there are children that present with larger tumors and metastatic disease that have a poorer outcome. Due to the rarity, there are still unanswered questions for this disease, such as:

• Are there children that may not need chemotherapy after successful surgical resection or transplantation?
• Is there a genetic trigger that can transform a benign mesenchymal hamartoma into a UESL?
• What is the optimal therapy for children with metastatic UESL?
• Are there predictive factors that could guide intensity of chemotherapy after surgery?

The North American Undifferentiated Embryonal Sarcoma of the Liver Registry was established at Texas Children’s Hospital to find answers to these questions and improve outcome of children with this rare form of liver cancer.

Registry goals

• To enroll all children diagnosed with undifferentiated embryonal sarcoma in North America and collect clinical and biological information
• To serve as a resource for patients and their families, and treating physicians to ensure optimal treatment of children with this rare form of liver cancer
• To serve as a resource for researchers involved in pediatric liver cancer research

How to enroll

If you or your child is diagnosed with undifferentiated embryonal sarcoma of the liver and you are interested in finding more information about this registry, please contact us by emailing raretumors@texaschildrens.org or by calling 832-824-6835.

Registry personnel

Sanjeev A. Vasudevan, MD, Pediatric Surgical Oncologist
Prakash Masand, MD, Pediatric Radiologist
Dolores Lopez-Terrada, MD, PhD, Pediatric Pathologist
Andras Heczey, MD, Pediatric Oncologist
Rajkumar Venkatramani, MD, MS, Pediatric Oncologist
Josalind Randall, Research Coordinator