Most Common Pediatric Brain Tumor

Brain Tumors By Race/ethnicity*

• Overall, Black / African American people have slightly higher incidence rates of primary brain and other CNS tumors compared to other races at 24.58 per 100,000 persons in the U.S., followed by:
• White 24.24/100,000
• Asian and Pacific Islander American 19.52/100,00
• American Indian and Alaskan Native 14.63/100,000

Astrocytomas Including Glioblastoma Multiforme

Astrocytomas are the most common type of glioma, accounting for about half of all childhood brain tumors. They are most common in children between the ages of 5 and 8.

The tumors develop from glial cells called astrocytes, most often in the cerebrum , but also in the cerebellum .

The grade of an astrocytoma is important. Your childs treatment will be based on whether or not the tumor is slow-growing or fast-growing . Most astrocytomas in children are low-grade. Sometimes they begin in the spine or spread there.

There are four main types of astrocytomas in children:

Pilocytic astrocytoma : This slow-growing tumor is the most common brain tumor found in children. Pilocytic astrocytoma is often cystic . When this tumor develops in the cerebellum, surgical removal is often the only treatment necessary. Pilocytic astrocytomas growing in other locations may require other therapies.

Diffuse astrocytoma : This brain tumor infiltrates the surrounding normal brain tissue, making complete surgical removal more difficult. A fibrillary astrocytoma may cause seizures.

Anaplastic astrocytoma : This brain tumor is malignant. Symptoms depend on the location of the tumor. These tumors require a combination of treatments.

Glioblastoma multiforme : This is the most malignant type of astrocytoma. It grows rapidly, and often causes pressure in the brain. These tumors require a combination of treatments.

How Are Brain Tumours Treated

Brain tumour treatment will vary according to the precise type of tumour, its grade, position and the extent to which it may have spread. Doctors will carry out a range of tests in order to obtain this information, including taking a biopsy and the use of a CT or MRI scan.

The main treatment for most brain tumours in children is surgery . If a tumour cannot be completely removed surgically, or if there is a chance that some cancer cells have been left behind, radiotherapy may be given afterwards. Radiotherapy may also be used as the main treatment if surgery is not possible, however it is usually avoided in children under the age of three years, as there is a greater risk of harm to the developing brain.

Chemotherapy is used routinely to treat medulloblastoma and is increasingly being used to treat other types of brain tumours. It may also be used in situations where the tumour cannot be removed completely with surgery.

The child may be given steroids. These do not treat the tumour but can improve symptoms by reducing the swelling that often surrounds brain tumours. Anti-convulsants may also be given to prevent fits.

Other possible treatments may be considered such as proton beam therapy . Higher doses of chemotherapy which kill the bone marrow cells as well can be given but with stem cell rescue .

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Atypical Teratoid Rhabdoid Tumor

These rare tumors of neuroectodermal origin are typically found in the cerebellum in very young children.

Although not common among children, other brain tumors that can occur include oligodendroglioma, orbital tumor, pituitary tumor, ganglioglioma, and meningioma. Despite being rare among children generally, meningioma is common among those children who have the genetic condition neurofibromatosis type 2.

Molecular Insights Current Status And Future Directions

While the above studies were in progress, a series of molecular analyses demonstrated that many pilocytic astrocytomas exhibit translocations or, less commonly, activating mutations of the BRAF gene, which may promote tumor development .26,48BRAF–KIAA fusions are common in cerebellar and optic pathway pilocytic tumors and lead to constitutive activation of the BRAF protein, whereas BRAF mutations are more common in gangliogliomas, pleomorphic xanthoastrocytomas, and cerebral pilocytic astrocytomas .61 Tumors lacking BRAF fusions or mutations often have alterations in other components of the mitogen-activated protein kinase signaling pathway, including NF1 mutations and RAF fusions. This convergence of mutations on a single downstream pathway prompted interest in the targeted inhibition of MAPK signaling as a therapy for these tumors. Recent studies using agents that inhibit MAPK activation by blocking MEK1/2 , such as selumetinib,2 have had promising initial results. In a Pediatric Brain Tumor Consortium phase I study of this agent, 5 of 25 LGGs had durable partial responses, and the majority had at least some tumor shrink-age.2 Based on these results, a phase II study of this agent was launched, which stratified patients by MAPK pathway mutation status , histological diagnosis, and presence of NF1. Given the strong activity observed in several of these strata, new clinical trials are already incorporating MEK inhibitors alone or in combination for newly diagnosed patients.

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Tests For The Child With A Brain Tumor

In putting together the most appropriate treatment plan for a child with a brain tumor, the neurosurgery team will need to know:

• Tumor location: This is determined by a brain scan, using one or more types of imaging such as CT or MRI. Because there are many vital structures in the brain, there are places a tumor can grow that are not appropriate for surgery. The neurosurgeons careful evaluation will determine the accessibility of the tumor and the safest approach.
• Brain tumor type: Looking at the tumor cells under a microscope can reveal the brain tumor type, and give doctors insight on how the tumor is likely to grow or spread.
• Brain tumor grade: The grade refers to how aggressive the tumor cells appear to be. The higher the grade, the more aggressive the tumor.

Central Nervous System Primitive Neuroectodermal Tumors

Primitive neuroectodermal tumors of the CNS are predominantly hemispheric tumors that account for approximately 3% to 5% of all pediatric brain neoplasms.2 This group is histologically and molecularly heterogeneous with variable neuronal, ependymal, or glial differentiation. The World Health Organization currently classifies CNS-PNETs as supratentorial PNETs, neuroblastoma, medulloepithelioma, and ependymoblastoma.63 The biologic relationship between these various histologic and anatomic categories remains unclear. Traditionally, the treatment for these tumors has been similar to that for other embryonal tumors, such as medulloblastoma however, patients with CNS-PNETs fare poorly despite intensified treatments. Comprehensive and integrative profiling of a large number of CNS-PNETs, in which gene expression and copy number profiles were combined, have uncovered three distinct subgroups with distinct expression and clinical signatures: primitive-neural , oligoneural , and mesenchymal .64

Daphne A. Haas-Kogan MD, … Paul Graham Fisher MD, in, 2010

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Brain Tumors In Children

• 13,657 children are estimated to be living with a primary brain tumor in the U.S.
• Approximately 4% of all brain tumors cases diagnosed each year occur in children ages 0-14
• An estimated 4,170 new cases of childhood brain tumors are expected to be diagnosed in 2022
• Brain tumors are the most common solid cancer in children ages 0-14 years
• The five-year relative survival rate for all primary childhood brain tumors is 82.8%
• For malignant tumors, the five-year survival rate is 74.8%

Atypical Teratoid Rhabdoid Tumor Supratentorial Primitive Neuroectodermal Tumor Pineoblastomas And Central Nervous System Germ Cell Tumors

Atypical teratoid rhabdoid tumors , supratentorial primitive neuroectodermal tumors , pineoblastomas, and non-germinomatous germ cell tumors of the central nervous system are similar tumors to medulloblastomas, but can occur in all parts of the brain. They frequently require CSI and therefore derive similar advantages from proton therapy. When these tumors occur outside the fourth ventricle, proton therapy may be recommended to spare the brains developing intellectual regions, hormone centers, and pathways controlling vision and hearing. ATRT is a particularly difficult tumor to treat with conventional radiation because it most often occurs in infants and requires intensive chemotherapy in addition to radiation. There is growing evidence that proton therapy offers a less damaging approach to treating these infants, particularly in patients who have undergone a complete surgical resection. In 2018, researchers from the University of Florida published the results from their experience treating NGGCT in children, the largest series to date involving proton therapy.2

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Where To Find Clinical Trials

The Pediatric Brain Tumor Consortium lists institutions that treat pediatric brain tumors:

The treatment centers listed below under Resources, Web sites often list their own clinical trials.

Clinical trials for brain tumors can also be found by searching the clinical trial database on the NCI web site. Follow these directions:

• Choose type of cancer: brain tumor, child, type. You will be given more choices : choose the appropriate responses and click search.

Are There Side Effects

Brain and spinal cancer treatment often causes side effects, these will depend on the exact treatment being given but may include:

These problems are temporary and can be minimised with good supportive care. Treatment can also cause long-term effects, outlined under Follow-up below.Side effects of treatment

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What Causes Pediatric Brain Tumors

The cause of primary pediatric brain tumors remains largely unknown.

Some of the possible risk factors for developing a brain tumor as a child include radiation exposure, or having an inherited condition. Some inherited conditions put a child at increased risk for certain types of brain tumors. The following inherited conditions may cause pediatric brain tumors: neurofibromatosis types 1 and 2, tuberous sclerosis, Von Hippel-Lindau disease, Li-Fraumeni syndrome, and other syndromes.

What You Need To Know

• Brain tumors are the most common solid tumors affecting children and adolescents, with close to 5,000 children diagnosed each year.
• Because of their location, some pediatric brain tumors and their required treatments can cause significant long-term impairment to intellectual and neurological function.
• Some forms of brain cancer can be life-threatening.

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What Are The Symptoms Of A Brain Tumor

The following are the most common symptoms of a brain tumor. However, each child may experience symptoms differently. Symptoms vary depending on size and location of tumor. Many symptoms are related to an increase in pressure in or around the brain, except in very young children , as there is no spare space in the skull for anything except the delicate tissues of the brain and its fluid. Any tumor, extra tissue, or fluid can cause pressure on the brain and result in the following symptoms:

• Increased intracranial pressure . ICP is caused by extra tissue or fluid in the brain. Pressure may increase because one or more of the ventricles that drain cerebrospinal fluid has been blocked, causing the fluid to be trapped in the brain. Increased ICP can cause the following:

The symptoms of a brain tumor may resemble other conditions or medical problems. Some pituitary tumors may cause diabetes insipidus, with symptoms of abnormal electrolytes and changes in mental status. Some people with deep thalamic tumors have abnormal eating patterns. These tumors may be particularly difficult to diagnose in adolescents because of the symptoms. Always consult your child’s doctor for a diagnosis.

How Is A Tumor In The Brain Evaluated

When a tumor is suspected, the first step is neuroimaging. Usually MRI scanning with and without a contrast agent is the preferred study. The contrast agent is a drug given intravenously which can provide additional detail of the tumor and surrounding nervous system structures. Once the MRI is completed, treatment decisions are made. Usually a surgical biopsy or resection is performed. Pathologic evaluation of the tumor directs subsequent treatment choices, which include chemotherapy and/or radiation therapy.

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Symptoms Of A Pediatric Brain Tumor

Because the human skull has no excess room for anything other than protecting the brain, it cannot accommodate the size and spreading of a brain tumor. Eventually, the extra pressure in this closed space can block the cerebrospinal fluid flow pathways and impede the functions the brain carries out.

Symptoms of pediatric brain tumors vary, but generally include:

• Severe, chronic headaches

• Enlarged head, especially in infants whose skull bones were not properly fused

Histopathology And Molecular Pathology

Neuropathology of pediatric brain tumors has changed significantly as a result of deeper understanding of molecular biology. Although the most recent 2007 WHO classification is still based predominantly on histopathology alone and pediatric HGGs are embedded within the adult HGGs, the molecular data indicate that adult and pediatric HGGs are biologically distinct.

In children, two main histopathological and clinical variants of HGGs are recognized. Whereas supratentorial glioblastoma multiforme is histologically, albeit not molecularly, similar to its adult counterpart, DIPGs can range in histologic features from diffuse astrocytoma, WHO grade II, to GBM, WHO grade IV, despite aggressive behavior across all grades. Histologic criteria show little, if any, correlation with clinical outcome.

Most pediatric HGGs develop as a result of somatic mutations. The number of somatic mutations in children is relatively low compared with adult GBM however, it is higher than in many other pediatric cancers, with a median of 15 nonsynonymous coding mutations per sample in both DIPGs and noninfant HGGs. The genomic complexity of pediatric HGGs spans a wide range, with infant HGGs showing extremely low numbers of mutations, with a median of 2. On the opposite side of the spectrum are HGGs from patients with mismatch repair abnormalities, which show the highest mutational burden of all tumors, with a median of 6810 nonsynonymous coding mutations per sample .

Stephan D. Voss, in, 2009

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Types Of Brain Tumors In Children

Brain tumors can be categorized as:

Primary: Starting in the brain

Metastatic: Starting in other parts of the body and spreading to the brain

Benign: Slow-growing non-cancerous. Benign tumors can still be difficult to treat if they are growing in or around certain structures of the brain.

Malignant: Cancerous. Unlike benign tumors that tend to stay contained, malignant tumors can be very aggressive. They grow rapidly and can spread to areas near the original tumor and to other areas in the brain.

Types Of Brain Tumors

A brain tumor, known as an intracranial tumor, is an abnormal mass of tissue in which cells grow and multiply uncontrollably, seemingly unchecked by the mechanisms that control normal cells. More than 150 different brain tumors have been documented, but the two main groups of brain tumors are termed primary and metastatic.

Primary brain tumors include tumors that originate from the tissues of the brain or the brain’s immediate surroundings. Primary tumors are categorized as glial or non-glial and benign or malignant.

Metastatic brain tumors include tumors that arise elsewhere in the body and migrate to the brain, usually through the bloodstream. Metastatic tumors are considered cancer and are malignant.

Metastatic tumors to the brain affect nearly one in four patients with cancer, or an estimated 150,000 people a year. Up to 40 percent of people with lung cancer will develop metastatic brain tumors. In the past, the outcome for patients diagnosed with these tumors was very poor, with typical survival rates of just several weeks. More sophisticated diagnostic tools, in addition to innovative surgical and radiation approaches, have helped survival rates expand up to years and also allowed for an improved quality of life for patients following diagnosis.

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Treatment For Brain Tumors

If your child has been diagnosed with a brain tumor, you may want to consider getting a second opinion before beginning treatment. In fact, some insurance companies require a second opinion for such diagnoses. According to the American Cancer Society, it is very rare that the time it will take to get a second opinion will have a negative impact on your treatment. The peace of mind a second opinion provides may be well worth the effort.

Specific treatment for brain tumors will be determined by your child’s doctor based on:

• Your child’s age, overall health, and medical history

• Type, location, and size of the tumor

• Extent of the disease

• Your child’s tolerance for specific medications, procedures, or therapies

• Expectations for the course of the disease

• Your opinion or preference

Treatment may include :

• Surgery. Surgery is usually the first step in the treatment of brain tumors. The goal is to remove as much of the tumor as possible while maintaining neurological function. Surgery for a biopsy may also be done to examine the types of cells the tumor is made of for a diagnosis. This is frequently done if the tumor is in an area with sensitive structures around it that may be injured during removal.

• Antiseizure medication

• Ventriculoperitoneal shunt . A VP shunt may be placed in the head to drain excess fluid from inside the brain to the abdomen. A VP shunt helps control the pressure inside the brain.

• Bone marrow transplantation

• Supportive care

• Continuous follow-up care