Undifferentiated embryonal sarcoma of the liver: what families need to know | CION Cancer Clinics

Undifferentiated embryonal sarcoma of the liver is a rare, fast-growing cancer that starts in the liver's supporting tissue rather than in the liver cells. It is found mostly in older children and teenagers, and occasionally in young adults. Doctors often shorten the name to UESL or embryonal sarcoma.

What the long name actually means

"Undifferentiated" means the cells under the microscope do not look like any one normal tissue. "Embryonal" means they look young and primitive, like cells from early development. "Sarcoma" means a cancer of connective tissue. The word embryonal does not mean anything happened during pregnancy, and it does not mean a parent passed anything on.

How it differs from the liver cancers families have heard of

It is not hepatitis-related liver cancer, and it is not caused by alcohol, fatty liver or anything the child ate. The liver around it is usually healthy. It is also different from hepatoblastoma, the commonest liver cancer in young children, which has its own treatment plan; see hepatoblastoma in children.

The usual plan combines two things: surgery to remove the whole tumour, and chemotherapy before it, after it, or both. Results have improved a great deal since teams began using both together, which is why families should not be put off by older reports of a very poor outlook.

Surgery

Removing all of the tumour with a clear rim of healthy liver is the most important single step. CION's surgical oncologists assess and plan liver operations, and your team will tell you where the operation takes place. When the tumour cannot be removed safely, a liver transplant has been used for some patients at licensed transplant centres.

Chemotherapy

The drugs used are similar to those for other sarcomas in children and young people. They are standard medicines available in India, given at CION centres or by the paediatric team. Radiation therapy is used only occasionally, for tumour left behind after surgery.

It cannot tell you whether the lump on a scan is this tumour, a cyst or something else. It cannot tell you how your child or family member will do. Embryonal sarcoma is rare, the published experience is small, and the team that has seen the scans and the slides is the only one that can talk about outlook honestly.

Who this page does not apply to

It does not apply to a baby or toddler with a high AFP and a liver mass, which is far more often hepatoblastoma. It does not apply to an adult with cirrhosis or hepatitis B, where HCC is much more likely. For other sarcomas found in adult livers, see sarcoma in the liver.