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Doctors at Hassenfeld Children’s Hospital at ٺƵ diagnose and manage many types of tumors of the central nervous system, which includes the brain and spinal cord, in children. Some tumors are malignant, or cancerous, but most are benign, or noncancerous.
A brain tumor is generally located in one of three areas—the cerebrum, the brainstem, or the cerebellum. The cerebrum consists of the right and left cerebral hemispheres, which are responsible for movement, sensation, thinking, and personality. The brainstem connects the brain with the spinal cord and the body’s nerves, and controls heartbeat, blood pressure, breathing, and swallowing. The cerebellum, in the back of the head, consists of the cerebellar hemispheres, which control balance and coordination.
Benign brain tumors rarely spread, but they can grow. They may press against nearby parts of the brain and invade healthy tissue in the area. Whenever possible, the primary treatment for benign childhood brain tumors is surgery to remove the tumor.
Malignant brain tumors can grow more quickly and spread to other areas of the brain and spinal cord, sometimes through the cerebrospinal fluid that circulates in and surrounds the brain and spinal cord. However, they rarely spread beyond the central nervous system. Treatment for malignant childhood brain tumors typically includes a combination of surgery, chemotherapy, and radiation therapy.
Many brain tumors grow by displacing, rather than invading, nearby brain or spinal cord tissue. Our pediatric neurosurgeons and neuro-oncologists are experts in diagnosing and treating the different types of central nervous system tumors in children.
Gliomas are the most common types of brain tumors in children. A glioma forms as a result of uncontrollable growth in glial cells, the brain’s most common type of cell. Glial cells are known as the support cells of the brain because they help nerve cells function. A glioma may be noncancerous or cancerous, and it may be slow growing, or low grade, or fast growing, or high grade.
Unlike gliomas in adults, the vast majority of childhood gliomas are benign tumors that can often be cured with surgical removal. In children, benign gliomas are twice as common as cancerous gliomas.
Though these slow-growing tumors can occur anywhere in the brain and spinal cord, the majority appear in the cerebral and cerebellar hemispheres. Children are more likely than adults to have these tumors in the brainstem; in the optic nerve, which transmits information from the eye to the brain; or in the hypothalamus, the part of the brain that controls mood and sleep.
The most common childhood glioma is juvenile pilocytic astrocytoma, or JPA. This slow-growing tumor develops from star-shaped brain cells called astrocytes, which surround and protect nerve cells in the central nervous system. Other types of benign gliomas include other low grade astrocytomas, gangliogliomas, dysembryoplastic neuroepithelial tumors, or DNTs, and desmoplastic infantile gangliogliomas, or DIGs.
Although total removal of benign tumors is curative, some slow-growing tumors develop in parts of the brain where they cannot be totally removed through surgery. These benign tumors may be treated with chemotherapy or radiation therapy.
High grade gliomas account for 8 to 12 percent of childhood brain tumors and include glioblastoma multiforme, anaplastic astrocytoma, and pontine (brainstem) glioma. Symptoms such as headache or nausea generally develop very quickly, often just weeks before diagnosis.
A group of malignant tumors that occur mainly in the cerebellum, central nervous system primitive neuroectodermal tumors, or PNETs, are usually large at diagnosis and fast growing. They’re called “primitive” because they arise from brain cells left over from the earliest stages of brain development in the womb. They are more common in younger children than in older children, and account for one in five childhood brain tumors.
This is the most common cancerous primitive neuroectodermal tumor, accounting for about 18 percent of childhood brain tumors. It occurs in the cerebellum and typically causes difficulty with balance or coordination. It may also lead to headache and sleepiness resulting from hydrocephalus, a blockage of the fluid pathways in the brain. This tumor can spread to other parts of the brain and the spinal cord, and it is most common in children under age 10.
Atypical teratoid–rhabdoid tumor is a rare, aggressive cancer. It most often occurs in the cerebellum, can be found anywhere in the brain, and frequently spreads to the spinal cord. It is most often seen in children age 2 or younger. It is sometimes caused by a gene mutation, in which the genetic material in the body’s cells changes, and may be inherited. It accounts for 1 to 2 percent of childhood brain tumors.
Choroid plexus papilloma occurs most commonly in children under age 2. It accounts for 3 percent of brain tumors in children and up to 20 percent of brain tumors in babies. The tumor develops from the choroid plexus within the ventricles, or fluid spaces, in the center of the brain. These tumors are often very large and can cause hydrocephalus—a buildup of fluid in the brain’s ventricles.
Most choroid plexus papillomas are slow growing and can be cured through surgery. Choroid plexus carcinomas, much less common, are faster growing and more likely to spread.
These rare tumors are usually located in “midline structures” in the brain—that is, those above the pituitary gland and near the pineal gland. The tumors can spread to other areas of the brain, as well as the spinal cord. They account for less than 4 percent of all childhood brain tumors.
These tumors arise from primitive cells formed in the womb that are designed to become gonadal, or reproductive, cells. Some of these cells can form tumors in the central nervous system; others can form germ cell tumors in the testicles in boys, the ovaries in girls, and elsewhere in the body.
Germ cell tumors of the central nervous system are usually diagnosed during adolescence and affect boys more often than girls.
Ependymomas are the sixth most common brain tumors in children and are often diagnosed in children under age 3. This malignant tumor arises from ependymal cells, which line the brain’s ventricles and the center of the spinal cord. These cells are responsible for making cerebrospinal fluid. Treatment typically includes surgery followed by radiation.
Craniopharyngioma is a slow-growing, benign tumor that develops near the brain’s pituitary gland, which controls hormone balance in the body; the optic nerves, which control vision; and the hypothalamus, which regulates many body functions. Most common in children ages 5 to 10, craniopharyngioma can increase pressure on the brain, interfere with the pituitary gland’s functioning, or damage vision. They account for 5 to 10 percent of all childhood brain tumors.
Symptoms can include a loss of vision, growth problems, frequent urination, headaches, nausea, and vomiting. Sometimes the tumor can affect behavior and learning.
This often slow-growing tumor arises mainly in the membranes that cover the brain, called meninges, but it can appear in the spinal cord. Most meningiomas are benign, but a small number are malignant. Meningiomas typically cause no symptoms when they are small and are often discovered incidentally through brain imaging after a trauma, such as a car accident.
As these tumors grow they can cause difficulties with finding words, seizures, vision problems, or weakness on one side of the body, depending on where the tumor is located. They are rare in children and more common in adults, particularly in women.
This typically slow-growing and benign tumor forms in tissue made from Schwann cells, which insulate nerves in the head and neck.
Symptoms depend on the location of the affected nerves and may include a lump, numbness, weakness, shooting pain down the arms or legs, or paralysis. Vestibular schwannomas, which form on the nerves to the ear, can cause a buzzing or ringing in the ear, hearing loss in one ear, and a loss of coordination or balance. This type of schwannoma may also affect the facial nerve, causing numbness or paralysis in the face.
Schwannomas are sometimes seen in children with neurofibromatosis, a genetic condition that causes tumors to develop in the central nervous system.