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About Rare Brain and Spine Tumors

Over-the-shoulder shot of clinician at a computer screen looking at brain MRIs

Who treats central nervous system tumors?

People with central nervous system (CNS) tumors are usually cared for by a team of healthcare professionals who have special training. In most cases, and depending on your needs, the following providers will be part of your care team: 

  • Neuro-oncologist: A doctor who specializes in the diagnoses and treatment of CNS tumors and other neurologic complications of cancer.
  • Oncologist: A doctor who treats cancer, including CNS tumors.
  • Neurologist: A doctor who manages disorders of the CNS.
  • Neurosurgeon: A doctor who diagnoses and surgically manages CNS diseases.
  • Radiation Oncologist: A doctor who plans and gives radiation therapy.
  • Neuroradiologist: A doctor who diagnoses diseases of the CNS using imaging techniques such as MRI, CT scan, and PET scan.
  • Neuropathologist: A doctor who examines CNS tissue to determine diagnosis.
  • Neuropsychologist: A psychologist who studies the function and structure of the brain and the thinking, memory, and behavior changes that can result from problems in the brain.
  • Psychologist: A healthcare professional who evaluates both normal and abnormal mental states and behavior.
  • Social Worker: A healthcare professional who helps people cope and resolve problems in their everyday lives by providing guidance and resources.
  • Genetic Counselor: A healthcare professional who evaluates risk and management of inherited conditions for people and families.
  • Nurse Practitioner and Physician Assistant: A healthcare professional who diagnoses and manages acute and chronic illnesses.
  • Nurse: A healthcare professional who cares for people, families, and communities, and oversees the management of care as prescribed by a doctor, nurse practitioner, or physician assistant. Nurses focus on inpatient care, outpatient care, hospice care, or management of research studies, as examples. 

People with a rare CNS tumor should seek a medical diagnosis and disease care and treatment with a team experienced in their disease.

Brain and Spine Tumor Treatment Advances

How are tumors monitored?

People with CNS tumors are usually monitored with imaging procedures. The most common are magnetic resonance imaging (MRI), which may include a specialized MRI such as spectroscopy. Sometimes computed tomography (CT) scans and positron emission topography (PET) are also used. How often you have imaging studies will be decided by your physician. A neuroradiologist will read your results from your scans and share them with your physician.

Who reviews tumors to determine an accurate diagnosis?

A neuropathologist should review your tumor tissue. The neuropathologist will determine the grade and type of tumor based on how it looks under the microscope.  He or she will also describe your tumor based on genetic changes and share the information with your physician.

How are tumors graded?

Primary CNS tumors are grouped by how the tumor tissue looks under a microscope and, sometimes, by certain genetic changes that may be present in the tumor. CNS tumors are graded based on World Health Organization (WHO) criteria as grade 1, grade 2, grade 3 or grade 4 (also written as grade I, II, III, or IV).  Some tumors are low grade tumors (grade 1 and grade 2) and others are high grade (grade 3 and 4).  Treatment and prognosis of primary CNS tumors depends on tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery. 

  • Grade 1: The tumor cells look more like normal cells under a microscope and grow and spread more slowly than grade II, III, and IV tumor cells. They rarely spread into nearby tissues. Grade I CNS tumors may be cured if they’re completely removed by surgery.
  • Grade 2: The tumor cells grow and spread more slowly than grade III and IV tumor cells. They may spread into nearby tissue and may come back as a higher-grade tumor.
  • Grade 3: The tumor cells look very different from normal cells under a microscope and grow more quickly than grade I and II tumor cells. They’re likely to spread into nearby tissue.
  • Grade 4 (high-grade): The tumor cells don’t look like normal cells under a microscope and grow and spread very quickly.

What is a tumor type?

CNS tumors are grouped together based on either the type of cell they start from or the location in the CNS. Subtypes are then defined within each tumor type, based on specific tissue characteristics or changes in the DNA. The tissue characteristics include:

  • How the cancer cells look under a microscope.
  • If there are certain substances in or on the cells.
  • If there are certain changes to the DNA of the cells.

There are over 100 tumor subtypes. It’s important to know your tumor subtype, if available, in order to plan treatment and determine prognosis. Ask your physician if your tumor has subtypes and how your tissue can be evaluated.

What is prognosis?

You may have questions about how serious your cancer is and your chances of survival. The likely outcome of your disease or your chance of recovery is called prognosis. Many factors can affect your prognosis. This includes your tumor grade and type, traits of your cancer, your age and health when diagnosed, and how you respond to treatment.

Doctors estimate prognosis by using statistics that researchers have collected over many years about people with the same type of cancer. Statistics are based on large groups of people. They cannot be used to predict exactly what will happen to you. If you want to understand your prognosis, talk to your doctor.

NCI-CONNECT partnered with the Central Brain Tumor Registry of the United States (CBTRUS) to understand the prevalence (number of people currently living with the disease), incidence (number of new people diagnosed each year), and survival rates of 12 selected rare primary CNS tumors. View the data.

Are CNS tumors genetic?

All cancers are genetic diseases. This means they are caused by certain changes in genes that control the way cells function. These changes often happen when cells grow and divide. Most cancers are caused by changes in the genes in the tissue or organ where they develop. Only 5 to 10% of all cancers are hereditary, which means passed down through a family.

Are CNS tumors hereditary?

Most CNS tumors aren’t hereditary. The small percentage of CNS tumors that are hereditary are due to rare syndromes that make someone more likely to have specific cancers. Doctors can find people and families at risk for these conditions by offering genetic counseling and tests. If you are concerned that your family or medical history may increase your chances of developing cancer, talk to a genetic counselor. NCI-CONNECT offers patients with select rare CNS tumors a personalized meeting with a genetic counselor during clinic.

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