Supratentorial primitive neuroectodermal tumors (SPNETs) are brain tumors found mainly in children.
They are relatively rare tumors seen in approximately 10 – 40 kids each year in the United States. These tumors occur in the cerebrum, the largest area of the brain that is responsible for many critical functions such as thinking, speech, emotions, and problem solving.
They are called primitive because they originate from cells that have not yet divided into specialized cells. The term neuroectodermal refers to the cells that SPNETs develop from being the same cells that will become the baby’s nervous system.
SPNETs are highly malignant, fast growing, and can spread to the central nervous system. Their cause is not known, but research speculates that they are the result of genetic mutations.
Signs and Symptoms
The signs and symptoms of SPNETs vary depending on the size and location of the tumor. Many of the signs of SPNETs are also signs of many other ailments. Children displaying these signs should always see a doctor.
Patients who suffer from SPNETs can experience symptoms such as headaches that are worse in the morning, vision problems, weakness on one side of the body, balance issue, and personality changes. The child might display lower energy levels than normal and cry more than usual.
Diagnosis
SPNETs are diagnosed first by a medical history and a physical exam. Doctors will often conduct vision tests if the eyesight is being affected. Scans such as a CT scan, an MRI, and a chest x-ray are used to pinpoint the exact size and location of the tumor. Bone scan tests can tell whether or not the cancer has spread. Doctors will also often test the kidney and liver functions. SPNETs are staged using only two categories – average risk or poor risk.
Treatment
A team of doctors often treats children with brain tumors. This team is led by a pediatric oncologist and can include their family doctor and several specialists. Social workers, clergy, or psychologists are also often part of the team when children are involved. They help the child and the parents cope with the emotional side of the illness.
Treatment depends on the age of the child, the size and location of the tumor and the stage of the tumor. Generally children under the age of three are not given radiation therapy. Their brains are still developing and the radiation might interfere with that. Surgery is performed to remove as much of the tumor as possible. This is sometimes difficult because SPNETs are often large tumors.
After surgery patients often receive chemotherapy. Doctors also sometimes insert a shunt in the brain to reduce any pressure from the tumor. For children over age three the same treatment is usually used with the addition of radiation.
Prognosis
Unfortunately the prognosis is not very good for patients with SPNETs. Due to the size and location of these tumors they are difficult to remove completely. The five-year survival rate for children over age three is 50 – 60%. SPNETs can recur and if they do they are often fatal.
