What is Childhood Brain Tumors?

Pediatric brain tumors are the most common type of cancer in children that forms a solid mass, second only to leukemia as a cause of cancer in children. These tumors can be divided into two categories based on their location in the brain: supratentorial (above tentorium) and infratentorial (below tentorium) tumors. Also, they can be classified based on when they’re diagnosed, such as congenital brain tumors (CBT) found at birth or in the first 60 days of life, tumors found in infants (under 1 year old), and those found in older children.

The outcome for children with brain tumors depends on different factors, including the child’s age when diagnosed, the type of tumor, and how much of the tumor can be removed surgically. CBTs act differently and might have a different outcome than the same type of tumors in older children. For instance, high-grade gliomas, a type of tumor, usually have a slightly better outcome in very young children compared to older children.

Thanks to improvements in imaging, molecular biology, and genetics, pediatric brain tumors are being diagnosed earlier. This allows for better sub-grouping and targeted treatment. Sadly, brain tumors are the leading cause of death among all cancers in children.

This summary focuses on congenital group, specifically on the most common types in this category which are; teratoma, choroid plexus papilloma, desmoplastic infantile tumors (DIA/DIG), glioblastoma multiforme (GBM), and medulloblastoma.

What Causes Childhood Brain Tumors?

Despite advances in medical science, we still don’t know exactly what causes brain tumors. They seem to occur due to a mix of genetics and environmental factors. For instance, brain tumors have been associated with certain genetic predispositions, such as DICER1, Li-Fraumeni, and other conditions involving the nerves and skin like neurofibromatosis, tuberous sclerosis, and Von-Hippel Lindau.

Family history could also play a part in the development of brain tumors, since a number of studies have found a link between brain tumors and having siblings.

The age of parents when a child is born may also be a factor. The findings of some research suggest that children born to women over the age of 40 have a higher risk of brain cancer, specifically astrocytoma and ependymoma.

There’s been some suggestion that exposure to infections during childhood could be linked to brain cancer, but there’s still debate around this.

It’s also been observed that high-dose radiation can lead to brain cancers. Children who have undergone radiation treatment for leukemia are known to be at risk of developing brain cancer.

Understanding more about how brain tumors develop is a complex topic, and goes beyond this brief overview.

Risk Factors and Frequency for Childhood Brain Tumors

The occurrence of brain tumors in children varies in different parts of the world, from 1.15 to 5.14 cases per 100,000 children. In the U.S, this occurrence is the highest. Also, Congenital Brain Tumors (CBT) occur from about 0.3 to 2.9 cases per 100,000 live births worldwide. The prognosis and survival rates hinge on several factors, such as the subtype of the tumor and its location.

  • For children born with brain tumors, the most common type is a teratoma (26.6% to 48% of cases).
  • The second most common is astrocytoma (7.4% to 28.8% of cases).
  • Choroid plexus papilloma ranks third (3.7% to 13.2% of cases).
  • Embryonal tumors make up about 3% to 13% of cases.
  • Craniopharyngioma comes next with 5.6% to 6.8% of cases.
  • Lastly, ependymoma makes up around 4.4% of cases.

Signs and Symptoms of Childhood Brain Tumors

Choroid Plexus Brain Tumors, or CBTs, can occasionally be found during ultrasound scans in the second or third trimester of pregnancy. These scans may show signs like a mass in the brain, a condition where there is too much fluid in the brain (hydrocephalus), or too much amniotic fluid (polyhydramnios). To confirm these findings, doctors are using MRI scans more and more. After birth, the most common symptoms are hydrocephalus, an abnormally large head size (macrocrania), and specific neurological signs.

In babies and older children, the symptoms can differ based on the tumor’s location. Tumors located above the tentorium (a membrane in the brain) can lead to symptoms like weakness in the limbs, seizures, and changes in consciousness. Tumors in the lower part of the brain can often cause symptoms like a rise in pressure inside the skull and imbalance.

Testing for Childhood Brain Tumors

Teratoma is a type of tumor often spotted as a mixture of solid and fluid-filled areas within the brain during an ultrasound exam in the second or third trimester of pregnancy. MRI scans show a variety of colors, and the appearance of fat signals is usually a very clear sign of teratoma. CT scans show it as a mixed density lesion with often coarse calcification, and again, the presence of fat is a good indication of the diagnosis. These types of tumors are more commonly found in the upper part of the brain than the lower.

Glioblastoma multiforme is a severe type of brain tumor that usually shows up as a large, vague mass taking up most of one brain half or spread into the other half. Ultrasound images depict it as a varied echo-producing mass that often includes areas of bleeding and dead tissue. Restriction in the movement of water molecules, which can be identified using MRI, is an important characteristic that hints at the especially severe nature of this tumor. Symptoms of increased pressure inside the skull are common with this condition.

Choroid plexus papilloma, another type of brain tumor, is usually seen as an area of increased density within the brain’s fluid-filled spaces on a CT scan. On MRI, it appears less intense or equal to the intensity of brain tissue on T1 images and equal to or more intense on T2 images. This type of tumor often leads to significant fluid accumulation in the brain.

Medulloblastoma appears typically as a tumor located in the center of the lower part of the brain, growing into one of the brain’s fluid-filled chambers. On a CT scan, it appears denser than surrounding structures. An MRI shows different intensities on T1/T2 images, and may also show restriction on the movement of water molecules and varying contrast enhancement.

In all these cases, the entire nervous system needs to be scanned to check for the spread of cancer cells in the cerebrospinal fluid and spinal cord.

Treatment Options for Childhood Brain Tumors

The main way to treat CBT (Chordoid Glioma of the Third Ventricle) is through surgery, where all or as much of the tumor as possible is removed. The size of the tumor and the patient’s general health both play a big role in how successful the surgery may be. Additional treatments aren’t usually used for newborns. A treatment called craniospinal radiotherapy, which could seriously hinder a child’s development, is avoided for the first three years of life.

Brain Abscess

In newborn babies, a brain abscess (a pocket of pus in the brain) can sometimes develop due to bacterial meningitis. This happens in 1.3% to 4.0% of cases. Additionally, a group of infections known as TORCH (toxoplasmosis, rubella, cytomegalovirus, and herpes simplex) may look like diseases that have spread from tumors located outside the skull in children.

Bleeding in the Brain (Intracranial Hemorrhage)

Sometimes, bleeding in the brain can look like tumors in scans, especially during the intermediate and late stages. There might be changes in the collected blood and inflammation around it that show up on CT and MRI scans. Specific MRI sequences can help differentiate between the two and monitor progress. If a blood vessel abnormality is suspected, specialized MRI or CT scans of the blood vessels can be done to confirm.

Stroke

Strokes can also look like tumors in medical imaging. This can be especially confusing when the stroke causes signs similar to a tumor such as swelling or irregular accumulations of dye used during the scanning process. Advanced MRI sequences can provide additional information. Understandably, if the swelling restricts the diffusion of water, then the swelling might be due to a tumor. However, understanding the patient’s medical history and knowledge of the areas of the brain supplied by specific arteries and veins are critical to making the correct diagnosis.

Neurocutaneous Syndromes

The central nervous system (CNS) can be affected by several well-known genetic conditions that can lead to brain tumors. These include neurofibromatosis (NF), tuberous sclerosis (TS), Sturge-Weber syndrome, Von-Hippel Lindau (VHL), and hereditary telangiectasia. For instance, NF-1 could lead to low-grade brain tumors, while TS could be linked to a type of brain tumor called subependymal giant cell astrocytomas (SEGA). In the case of VHL, hemangioblastomas, another type of brain tumor, are common. It can be quite challenging to tell the difference between these genetic conditions and brain tumors, but understanding the specific patterns of these conditions can help doctors make the correct diagnosis.

What to expect with Childhood Brain Tumors

Brain tumors present from birth generally have a low survival rate, with less than 30% of individuals surviving. Several factors significantly impact these survival rates, including the severity of the tumor type, its size and location, the stage of fetal development when the tumor formed, and complications related to treatment.

Possible Complications When Diagnosed with Childhood Brain Tumors

Complications can change depending on several factors like the type of tumor, its location and size, as well as the treatment plan.

Preventing Childhood Brain Tumors

Successfully treating brain tumors heavily relies on teamwork. It’s essential to thoroughly educate families and involve them in the decision-making process when it’s suitable. Experts typically converge at multidisciplinary meetings to discuss the treatment plans, identify the best available treatment options, and forecast the patient’s health outcome. Providing emotional support and therapeutic counseling to the family during treatment can tremendously influence the whole journey. In specific cases, genetic testing and profiling are also made available to families. These tools can aid in determining the disease’s future outlook and help in planning the family’s future.

Frequently asked questions

Childhood brain tumors are the most common type of cancer in children, forming a solid mass in the brain. They can be divided into two categories based on their location in the brain and can also be classified based on when they are diagnosed.

The occurrence of brain tumors in children varies in different parts of the world, from 1.15 to 5.14 cases per 100,000 children.

Signs and symptoms of Childhood Brain Tumors can vary depending on the location of the tumor. Here are some common signs and symptoms: 1. In babies and older children, tumors located above the tentorium (a membrane in the brain) can cause: - Weakness in the limbs - Seizures - Changes in consciousness 2. Tumors in the lower part of the brain can often cause: - A rise in pressure inside the skull - Imbalance In addition to these location-specific symptoms, there are also general symptoms that may be present in children with brain tumors: - Hydrocephalus: This is a condition where there is too much fluid in the brain. It can cause symptoms such as headache, nausea, vomiting, and changes in vision. - Macrocrania: This refers to an abnormally large head size. It can be a sign of a brain tumor in infants. - Specific neurological signs: These can include changes in behavior, difficulty with coordination, problems with speech or hearing, and developmental delays. It's important to note that these signs and symptoms can vary from child to child, and not all children with brain tumors will experience the same symptoms. If you suspect that your child may have a brain tumor, it's important to consult with a healthcare professional for a proper diagnosis and appropriate treatment.

Childhood brain tumors can occur due to a mix of genetics and environmental factors, including certain genetic predispositions, family history, advanced parental age, exposure to infections during childhood, and high-dose radiation treatment.

A doctor needs to rule out the following conditions when diagnosing Childhood Brain Tumors: 1. Brain Abscess 2. Bleeding in the Brain (Intracranial Hemorrhage) 3. Stroke 4. Neurocutaneous Syndromes (such as neurofibromatosis, tuberous sclerosis, Sturge-Weber syndrome, Von-Hippel Lindau, and hereditary telangiectasia)

The types of tests needed for Childhood Brain Tumors include: - Ultrasound exam during pregnancy - MRI scans - CT scans - Scans of the entire nervous system to check for spread of cancer cells in the cerebrospinal fluid and spinal cord.

Childhood brain tumors, such as CBT (Chordoid Glioma of the Third Ventricle), are primarily treated through surgery. The goal of the surgery is to remove as much of the tumor as possible. The success of the surgery depends on factors like the size of the tumor and the patient's general health. Additional treatments are typically not used for newborns. However, a treatment called craniospinal radiotherapy, which can negatively impact a child's development, is avoided for the first three years of life.

The prognosis for childhood brain tumors depends on various factors, including the child's age at diagnosis, the type of tumor, and the extent of surgical removal. Congenital brain tumors (CBTs) have a lower survival rate, with less than 30% of individuals surviving. The prognosis also varies based on the subtype and location of the tumor.

A pediatric oncologist.

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