Brain & Spinal Tumors
Brain and spinal cord tumors represent the most common solid tumors seen in children and second most common group of pediatric cancers after the leukemias. Approximately 3000 children will be diagnosed with a brain or spinal tumor in the United States each year. Since the brain is made up of many different types of cells, many different types of brain tumors can occur.Most children who develop a brain tumor have no apparent risk factors for the development of a tumor. There are a small number of children who appear to have specific genetic risks for certain types of brain tumors.
The specific symptoms that a child may have can be quite variable and depend on the location of the brain tumor, how quickly the brain tumor has grown, whether the brain tumor is pushing on normal brain structures, or blocking the flow of spinal fluid from the brain down to the spine.
Some of the symptoms that can occur include:
• Nausea or vomiting
• Neck or back pain
• Problems with eye or face movements
This is only a small listing of the types of symptoms that might be seen
The diagnosis of a brain or spinal cord tumor usually is made when a child presents with symptoms that are concerning for a possible brain tumor. The first step is a good history and physical examination with particular attention to the examination of the nervous system and the visual system. If the history and physical are concerning, your doctor may order a scan of the brain or spine. Often a CAT scan is done first but the best study for making the diagnosis is an MRI of the brain and/or spine.
One a scan is done and it is determined that there is a mass in the brain or spine, the child is usually referred to a center that specializes in the care of children with brain and spinal cord tumors (see section on the Johns Hopkins Pediatric Neuro-Oncology program). In most cases, an operation is required to try and remove the tumor and to determine what type of tumor is growing.
If an operation is required then tumor tissue will be sent to the pathologist who will look at the tumor under the microscope to make a diagnosis. There are many different types of brain tumors but some of the common tumors seen in children include:
Embryonal tumors (sometimes called neuronal tumors or PNETs)
- Atypical Teratoid/Rhadboid Tumors
- a. Pilocytic astrocytomas (the most common brain tumor seen in children)
- b. Pilomyxoid astrocytoma (PMA) (More on the Johns Hopkins Pilomyxoid Astrocytoma (PMA) Registry)
- c. Fibrillary (infiltrating or diffuse) astrocytomas
- Low-grade fibrillary astrocytomas
- Anaplastic astrocytoma (AA)
- Glioblastoma multiforme (GBM)
- Choroid plexus tumors
- Germ-cell tumors
- Non-germinomatous germ cell tumors (NG-GCT)
Certain tumors that occur commonly in adults (for example meningiomas and central nervous system lymphomas) almost never occur in children.
The other tests that may be required depend on the type of tumor and the location. In some cases, aside from the MRI of the tumor, no further studies may be necessary. In other cases, further imaging studies may be required. In some cases, a spinal tap (also called a lumbar puncture) may be necessary to see if there are tumor cells floating in the spinal fluid. Usually it is unnecessary to get scans of other parts of the body besides the brain and spinal cord.
The approach to the treatment of a brain or spinal cord tumor depends on a number of issues including:
- How much of the tumor was removed at the time of surgery
- What type of tumor
- Whether the tumor has spread elsewhere
- Whether the tumor likes to spread elsewhere
- The age of the child
The three major types of treatment utilized include:
- Radiation therapy
Information about the treatment of any specific tumor is beyond the scope of this summary. What is recommended is that all children with a brain or spinal cord tumor receive treatment at a center that is expert in the management of these tumors in children.
Johns Hopkins experts were the first to identify a class of brain cancers in children called pilomyxoid astrocytoma or PMA. Kenneth J. Cohen, MD, MBA, director of pediatric neuro-oncology, coordinates the Pilomyxoid Astrocytoma (PMA) Registry to gather information to help refine treatments and develop new ones.
Neuro-Oncology (Brain and Spinal Cord Tumors) Program
The management of children with brain and spinal cord tumors is extremely complex. Pediatric brain cancers can be stubborn, and typically do not respond to traditional chemotherapy and radiation. in part because the brain is somewhat protected from medications (the blood-brain barrier), in part because surgery in the brain has to be quite refined to minimize injury to brain tissue, and in part because radiation treatment to the brain can cause injury to normal brain tissue. To successfully manage and treat children with these tumors it takes a team of practitioners specifically focused on this area. The multidisciplinary Pediatric Neuro-Oncology program at the Johns Hopkins Kimmel Cancer Center in Baltimore is noted for the comprehensive care that is provided to all children diagnosed with a brain or spinal cord tumor and their families. Each year our practitioners treat between 80-100 children affected by these cancers tumors. A similar number of children's cases are reviewed as second opinions sent from around the world.
Subspecialists from a variety of disciplines at Johns Hopkins participate in a weekly tumor pediatric neuro-oncology conference, where each child’s case is presented and discussed in-depth, and an individually tailored treatment plan is worked out. These conferences include experts from the following areas:
- Pediatric neuro-oncology
- Pediatric neurosurgery
- Pediatric radiation oncology
- Pediatric neurology
- Social work
Experts with the program have access to rich resources, such as:
- State-of-the-art neurosurgical technologies including 3D navigation systems and intraoperative MRI technology
- Radiation therapy technologies that allow for the most precise delivery of radiation in an effort to spare normal brain tissue from the impact of radiation treatment. These include intensity-modulated radiation therapy (IMRT), which allows physicians to manipulate radiation beams to the shape of a tumor, and gamma knife, which focuses precise beams of radiation directly to a tumor.
- Access to the most innovative clinical trials for children with newly diagnosed brain tumors as well as for those children requiring further treatment should the brain or spinal cord tumor return or progress
- Access to other services as required including pediatric neurology, neuro-ophthalmology and endocrinology
- Neuropsychological assessment to gauge the impact of the tumor and treatment on the ongoing development of the child
- Ready access to social workers, child life specialists and others who make certain that all aspects of the child’s life are attended to during and after treatment
Cure will not come for many children with brain and spinal cord tumors without studying these tumors in the laboratory and using that gained knowledge to identify and test new treatments. Some of our brain and spinal tumor experts also are laboratory scientists working on new discoveries to better treat children with brain and spinal tumors. Physicians with the program have access to researchers deep within the fields of neuroscience, neuropathology and tumor immunology, and work to bring new discoveries to patients as swiftly as possible.
Dr. Kenneth Cohen and colleagues are progressive in leading and participating in early-phase clinical trials offering patients access to the latest therapeutic advances. These trials include the newest molecularly targeted therapies, often being used for the first time in children with brain and spinal cord tumors.
Ongoing efforts include studying different mechanisms to deliver treatments to the brain, discovering new drug treatments for brain and spinal cord tumors, and learning how certain switches within the brain turn on tumor suppressor genes. These are genes that put the brakes on fast-growing cancer cells.
One unique program run by Dr. Cohen is the Pilomyoxid Astrocytoma (PMA) Registry. Johns Hopkins experts were the first to identify this variant of a low-grade brain tumor in children. They now coordinate the registry to gather information regarding the natural history and response to treatment for children with PMAs.
Dr. Cohen is a member of the brain tumor steering committee and co-chair of the high-grade glioma committee for the Children’s Oncology Group (COG).
Dr. Eric Raabe leads a laboratory team of graduate students, post-doctoral fellows and research assistants focused on finding new therapies for the most difficult to treat brain tumors. He is investigating metabolic changes downstream of key genetic changes in high-risk brain tumors. He has active research projects in poor-prognosis medulloblastoma, atypical teratoid/rhabdoid tumor, and aggressive glioma, including glioblastoma and recurrent pilocytic astrocytoma. He developed one of a handful of diffuse intrinsic pontine glioma (DIPG) cell lines, and is using this cell line in a collaborative effort through the COG to identify new therapies for DIPG, which is one of the most devastating childhood cancers. He hopes the research will lead to an understanding of what causes DIPG to form, and what allows DIPG to continue growing, in an effort to find medications that target the mutations.