Embryonal Tumor with Multilayered Rosettes (ETMR)
A rare brain tumor mostly affecting very young children.
What Is an Embryonal Tumor with Multilayered Rosettes (ETMR)?
An embryonal tumor with multilayered rosettes (ETMR) is a rare and aggressive type of brain tumor that primarily affects children under the age of 3 years. The tumors affect both boys and girls but may be slightly more common in girls.
ETMRs are highly malignant and can be difficult to treat. Because of this, survival is poor. Less than half of patients survive a year or more after diagnosis. Treatment typically involves surgery, chemotherapy and radiation. Outcomes can be challenging due to the aggressive nature of the tumors.
What Causes an Embryonal Tumor with Multilayered Rosettes (ETMR)?
The exact cause of ETMRs is not fully understood. Like many cancers, it likely results from a mistake within brain cells that have not turned into their final form yet (embryonal or fetal cells). Something goes wrong with the cells before they can become their destined type of brain cell. ETMRs can occur in various parts of the central nervous system including the cerebellum, cerebral hemispheres, ventricles, brainstem and spinal cord.
However, they can be found in other parts of the brain, where they may be mistaken for other types of tumors. They can spread through spinal fluid to other parts of the brain and spinal cord, but they do not often spread to other parts of the body.
Doctors do not know why these tumors develop. They've found that in most ETMRs, genes on an area in one specific chromosome (c19mc) get amplified. Amplified means the body naturally makes more copies of the ETMR genes. This change is thought to play a key role in the development and progression of ETMRs. In a small number of cases, these tumors have a DICER1 mutation or have changes in the way genes are turned on and off that are similar to ETMRs involving C19mc. Because ETMRs commonly occur in infants and very young children, some researchers propose that ETMRs may develop from primitive cells or stem cells.
What Are the Signs and Symptoms of an Embryonal Tumor with Multilayered Rosettes (ETMR)?
Symptoms of brain tumors depend on where they are in the brain, how big they are and the child's age (under age 3). When the tumors press on important parts of the brain, they may cause problems such as:
- Dizziness
- Trouble walking or talking
- Vision issues, such as seeing double
- Nausea or vomiting
- Headaches
- Seizures
- Tiredness (Fatigue)
- Irritability
- Failure to thrive
- Rapidly growing head size
- Cranial nerve palsy
These problems can be difficult to notice in young children who cannot describe them. It may also be hard to know if your child is having trouble with balance or language if your child is barely walking or talking yet.
If you are worried about your child’s symptoms or development, reach out to a physician. The sooner your child is diagnosed, the better the chances are of survival.
How Is an Embryonal Tumor with Multilayered Rosettes (ETMR) Diagnosed?
ETMRs can resemble other brain tumors on MRI/CT and even when viewed under the microscope.
To accurately diagnose ETMRs, doctors need to conduct additional testing. They look for a specific mutation on the tumor tissue obtained via a biopsy or surgery depending upon the location of tumor. ETMRs can be misdiagnosed if doctors do not conduct these tests on tumor tissue.
An ETMR diagnosis is a histological diagnosis. This means tumor tissue is required to make the diagnosis. At Nationwide Children's, we are able to perform genetic testing, as well.
How Is an Embryonal Tumor with Multilayered Rosettes (ETMR) Treated?
Your child’s care team will come up with the best care plan for your child based on his or her age and tumor location. There are many pieces to treatment.
Surgery
Surgery plays a crucial role in improving survival rates in children with ETMRs. Ideally, completely removing the tumor is the goal. In many cases, this is not possible if the tumor is very large or involves critical areas of the brain and spinal cord. In some situations, aggressive surgery may do more harm to the child. In such cases, it is ideal to try and safely remove as much of the tumor as we can. Sometimes, a second surgery will be considered to remove any remaining tumor tissue.
IV Chemotherapy
After surgery, intensive intravenous (IV) treatment with chemotherapy is very important. Most children who survive a ETMR have had IV chemotherapy. There are various ways that can be used to give chemotherapy. Chemotherapy may include various combinations of drugs including high dose chemotherapy with stem cell rescue.
Radiation Therapy
Radiation therapy also plays a significant role in treating ETMR, especially when surgical removal is not feasible or when there is leftover tumor after surgery. As ETMRs affect young children, radiation therapy for ETMR needs to be weighed against its potential impact on the developing brain. Because of this, radiation has traditionally not been recommended for children under age 3. It is important that radiation therapy is carefully planned and delivered to minimize damage to healthy tissues surrounding the tumor site. The decision to use radiation therapy, type of radiation (proton vs. photon) the timing of treatment, and the dosage are determined based on the child's age, the extent of the tumor and several other factors considered by the team.
What to Expect With ETMR
It can be very scary to have a child diagnosed with any type of brain tumor. Our neurosurgery and neuro-oncology teams will answer your questions and take time with your family to decide how you would like to move forward.
The type of tumor your child has will affect the treatment plan. In most cases, imaging and tissue sample are required before a diagnosis will be made and a care plan will be put together. Once the diagnosis is confirmed, we can discuss the success of different types of treatment available, as well as what your goals are for care.
When to Seek Help for ETMR
If you have been told that your child’s tumor cannot be treated, you may wish to seek a second opinion from one of our neuro-oncology experts.
Why Choose Nationwide Children's?
The Neuro-Oncology Program at Nationwide Children’s Hospital offers clinical excellence in treating children, adolescents, and young adults with brain and spine tumors. Your child will have access to doctors who are experts in different types of medical care. We call this multidisciplinary care. Depending on the specific tumor diagnosis and treatment plan, your team may include oncologists, neurosurgeons, radiation oncologists, clinical psychologists, rehabilitation medicine doctors, neurologists, endocrinologists, ophthalmologists, therapists (physical, occupational, speech, art, music), social workers, dietitians and/or pharmacists. These team members all work together with the family to provide the best care for the patient.
Cancer Clinical Research
Nationwide Children’s is a national leader in oncology research and clinical trials. There are many ongoing research studies aimed at improving outcomes for children and young adults with brain/spine tumors—understanding why these tumors develop and how to appropriately target them. We are members of all major pediatric brain tumor clinical trial consortia including Pediatric Brain Tumor Consortium (PBTC), Collaborative Network of Neuro-oncology Clinical Trials (CONNECT), Pacific Neuro-oncology Consortium (PNOC) and the Children’s Oncology Group (COG). We have many open clinical trials testing new drugs in difficult-to-treat tumors, including in DIPG.
Next Steps
Speak With Our Team of Experts
Our team would be happy to answer any questions or schedule an appointment.
Request an Online Second Opinion
If you are currently receiving care at another institution and would like a second opinion from experts at Nationwide Children's, we can help.
