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Visual Pathway and Hypothalamic Glioma

Childhood Visual Pathway Glioma is a type of brain tumor in which cancer (malignant) cells begin to grow in the tissues of the brain. The brain controls memory and learning, the senses (hearing, sight, smell, taste, and touch) and emotion. It also controls other parts of the body, including muscles, organs, and blood vessels.

Diagnosis

Like most cancer, childhood brain tumor is best treated when it is found (diagnosed) early. Based on the symptoms, a Computed Tomographic (CT) scan of your child's brain may be required. A Magnetic Resonance Imaging (MRI) scan, which uses magnetic waves to make a picture of your child's brain, also may be done.

Often, surgery is required to see whether there is a brain tumor and to tell what type of tumor it is. The doctor may cut out a piece of tissue from the brain and look at it under a microscope. This is called a biopsy.

There are many types of brain tumors in children and the chance of recovery (prognosis) depends on the type of tumor, where it is located within the brain, and your child's age and general health.

There are treatments for all children with visual pathway gliomas.

Symptoms

The most common presenting complaint is a visible or palpable mass. Congenital lesions may be noticed at birth by the examining physician or the parents. Birth-related swelling of the scalp may obscure a congenital lesion or cephalohematoma that will later become obvious. Newborns and infants with high-flow vascular malformations may have signs of high-output heart failure including failure to thrive or cardiac murmurs.

BRIEF ABOUT THE PROCEDURE

In children with a scalp or skull tumor, surgery is indicated for decompression of neural structures, curative or palliative resection, correction of disfiguring deformities, relief of pain, and biopsy of an unknown lesion. All respective procedures has to be planned in anticipation of a reconstructive procedure.

In many cases, the defect left by resection of a skull tumor may be repaired primarily with a split thickness bone graft taken from adjacent cranium. Accordingly, the scalp incision should be designed to allow maximum vasculanty as well as access to potential donor craniotomy sites. Despite its length, the standard coronal scalp incision is frequently the best choice. In situations in which poor wound or graft healing may be anticipated (e.g., when radiation or chemotherapy is indicated) its advisable to delay the reconstruction until healing conditions are optimized, which may help avoid the loss of both donor and recipient grafts.

The need for intradural exploration is usually apparent from preoperative imaging studies; most scalp or skull tumors do not demonstrate intradural extension. However, congenital lesions frequently have a definite connection to the underlying brain that must be appreciated. Failure to completely excise a dermal tract may allow growth of an intracranial dermoid cyst or result in delayed infection. In addition, adhesion of the brain to overlying dura has been reported to cause neurological symptoms analogous to spinal cord "tethering". Thus, serious consideration should be given to intradural exploration when transdural extension is apparent.

The various procedures adopted are:

  • Surgery (Removing the cancerous tissue through an operation)
  • Radiation Therapy (using high-dose X-Rays to kill cancer cells)
  • Chemotherapy (Oral or IV drugs to kill cancer cells)

The treatment will often be coordinated by a pediatric oncologist, a pediatric neurosurgeon (a specialist in childhood brain surgery), a pediatric neurologist, a psychologist, a radiation oncologist, and other doctors who specialize in the type of treatment the child requires.

Surgery is one treatment for visual pathway glioma. Depending on where the cancer is and the type of cancer, your child's doctor may remove as much of the tumor as possible. If the tumor cannot be totally removed, radiation therapy and chemotherapy may also be given. If the cancer is in a place where it cannot be removed, then surgery may be limited to a biopsy of the cancer.

Radiation therapy (external radiation therapy) uses high-energy X-Rays to kill cancer cells and shrink tumors. The use of radiation put into the brain through thin plastic tubes (internal radiation therapy) is under study. For some types of brain tumors, clinical trials are evaluating radiation therapy given in several small doses per day (hyperfractionated radiation therapy). Radiation therapy can affect growth and brain development, so clinical trials are testing ways to decrease or delay radiation therapy, especially for younger children.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body. Chemotherapy is being studied to delay the use of radiation therapy in some patients. Clinical trials are studying different chemotherapy drugs for visual pathway gliomas.

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