Primitive neuroectodermal tumor is a complicated form of cancer that occurs in the brain, most often in young children who are less than ten years old, but possibly older. The term tends to lead to misunderstanding because there are different ways of classifying these tumors. Usually classification occurs by location in the brain, which may vary. The reason such tumors are generally grouped together, no matter where they occur in the brain, is because they tend to be formed from cells that create brain matter and spinal matter, which are also called neuroepithelial cells. Since these are located in many places in the brain and the spine, symptoms of a primitive neuroectodermal tumor can be variable.
Some symptoms that might occur with a primitive neuroectodermal tumor include extreme headache, especially in the morning, tiredness or sleepiness, and bouts of nausea or stomach upset. Seizures could occur as a result of tumor growth, and additional symptoms might be present depending on where the tumor is located. There could also be changes to motor skills, vision, and mood. Occasionally a primitive neuroectodermal tumor occurs in the spine and this could affect functions of the lower body like the bladder and bowels.
When primitive neuroectodermal tumor is suspected, people are often referred to tertiary hospitals for more extensive testing. Early testing focuses on finding tumors through a variety of scanning techniques. These could include x-ray, magnetic resonance imaging (MRI) and computed tomography or CAT/CT scans.
Since these tumors are malignant they do spread. Once found, doctors needs to determine staging of the primitive neuroectodermal tumor, which is not that similar to staging in other forms of cancer. Instead doctors will identify these cancers by risk factor. Risk relates to location of tumor, how much can or is removed during surgery, age of child, and degree of spread of the cancer to other parts of the body. Higher risk, or what is called poor risk, is usually defined when the a tumor occurs in toddlers and especially if it has spread through the body or is in a part of the brain that makes it impossible to surgically remove all of it.
Surgical removal is the usual first treatment for this form of cancer and this is then followed with a variety of approaches to kill malignant cells and arrest cancer growth. These treatments could include radiation and/or chemotherapy. Even with initial successful treatment, there is risk for recurrence of tumors. This recurrence tends not to respond well to treatment and has extremely poor prognosis.
Primitive neuroectodermal tumor is fortunately extremely rare. While this is good from the perspective that few people will experience it, it also means there are few opportunities to conduct wide scale research on how to eradicate it. Treatment today cannot cure all cases, though general knowledge about treating cancer continues to improve and may be applied to these forms of tumors in the future.
