BRAIN TUMORS IN CHILDREN

"I am truly sorry, but your child has a brain tumor..."

The World of Childhood Brain Tumors has no "Welcome" sign leading into it.

No matter what anyone says to minimize the situation, this statement is the most painful sentence a human can hear. The combination of fear, shock, pain is much to bear.

"To see such innocence, so roughly shaken by this terrible trial, at such a young age, is nothing short of incomprehensible." I still hear this sentence, uttered by a visibly shaken parent. Instead of my becoming more routine and distant, the visceral terror that it engendered in me (the supposed all- knowing physician/advisor), the delivery of such information has grown more and more difficult, even personal. I watch my own small children grow and develop. As a physician, one realizes more than ever, there is no place for impersonal behavior when caring for the children who are afflicted by a brain tumor, benign or otherwise--their families as well. No amount of experience or training can help one to ease or minimize the situation.

When we discuss special areas such as:

• Outcome: Does "excellent long-term results" mean a ten-year survival for a 3-year old-- your child living long enough to be frustrated by death at the age of thirteen? We parents think only of 50 and 60 year plans. Five and ten-year plans are unacceptable. We want to know that our grandchildren will not live to see their parents die at a young age.
• Quality of life: Does that mean that a 5-year old child will have no hair, be skinny and slow to walk and play with his friends and classmates? How can we parents bear to live through the process of trying to give our child a childhood instead of a world of I.V. tubes, doctors who speak of life-and-death, hospital corridors and toys that our children will never play with?
• Doctors: Who are they? Can we do anything to keep them human, warm, and caring for our child? How do we deal with our own resentment for the fearful news, difficult therapies and their human frailty? How do we keep our child happy to see them? Most children cringe at the mere mention of visiting a doctor-with-a-shot-to-give, or a visit to the dentist with his drills and instruments?
• Hospitals: How do we keep these innocents away from such large and frightening monoliths?
• We parents: How can we possibly bear all of this without frightening our little, loving child?
• Hope: Yes, there IS hope! God and goodness are your guide. Your child is truly a precious gift. No matter what cards we are dealt, a child's joy and innocence are somehow never lost. In the worst of times, a child's pain often becomes the parent's teacher. And, when we expect the best for our children, we often get it despite all odds.

THE BIOLOGY OF CHILDHOOD BRAIN TUMORS

Pediatric brain tumors are different

Most frequently, they come from "young" cells. These are cells that are still developing ("immature" or "primitive" cells) and have not reached full maturity. They are developing at the same time as the child is developing. If one looks at the way a normal cell matures from its very beginning as a "primitive" brain cell (a precursor) through its stages of normal development, towards becoming an adult cell type, one can start to understand the logic of the progression of tumor types in children.

A diagrammatic representation of the manner in which brain cells develop from the embryo to the adult is under constructiion. It will be posted soon.

For every normal cell type, there is a corresponding tumor that can arise from it. Thus, there is the primitive neurectodermal tumor (PNET), the medulloblastoma, the astroblastoma, the neuroblastoma, the astrocytoma, the gangliocytoma/neurocytoma, the ependymoma and so on.

There are also other unusual tumors such as the teratoma, the pinealblastoma, the esthesioneuroblastoma, etc. There are also other tumors thay come from non-brain origins such as the pituitary tumors, teratomas, meningiomas, the skull bone tumors, and blood vessel tumors such as the hemangioblastoma or cavernous angioma.

Children commonly have brain tumors such as the PNET, medulloblastoma, various embryonic tumors and unusual tumors of the developing brainstem, hypothalamus and optic nerves (juvenile pilocytic astrocytomas, teratomas, etc.) There are variations of these tumors and they are rare as well as difficult to understand.

Parents and children should ask as many questions as possible. The questions can help reach level of comfort confident that the problem is clearly comprehended. Knowledge of the expected and the potentially unexpected will allow you to guide your child through the process of battle against their brain tumor. No matter how harried, tired or busy your surgeon and doctors might seem, it is important to understand the problem at hand fully. This will make the efforts of all involved more effective. More often than not, knowledge helps us cope with the developing situation better.

The most important point is that some of these tumors hold the promise for a true and complete cure! There is nothing more satisfactory than the complete removal of a dreadful sounding cerebellar microcystic astrocytoma or a hemangioblastoma. Families soar from the depths of despair to the heights of sublime elation when the smiling surgeon comes to the waiting room after a difficult surgery. The surgeon may not look tired at all. The smile-on-his-face says that he has just had the privilege of completely removing a tumor!

MEDULLOBLASTOMA

This tumor is probably the most common tumor of children. It arises from one of the "junior" cells of the developing brain, called the medulloblastoma. This tumor almost always grows in the middle of the cerebellum (the balance part of the brain, in the back, behind the brainstem). More often than not, small children are found to have the tumor only after it has caused secondary problems relating to blockage of the normal flow of cerebrospinal from the ventricles to the drainage system located along the outer surface of the brain (Obstructive Hydrocephalus). This results in headaches, visual problems, and decreased alertness. Often, parents will notice "sundowning", (the eyes are "stuck" in the downward gaze position) of the baby's eyes, due to local pressure on one of the brain's centers for eye control.

EPENDYMOMA

The ependymoma arises from the cells that line the internal surfaces of the brain. These cells line the fluid spaces of the brain (ventricular system) and are relatively few in number. Some of these cells are quite specialized, having little frond-like protruberances that move the cerebrospinal fluid as it permeates into the brain from the ventricular system and back again.

These tumors are rare. They are usually found on the internal surfaces of the brain and spinal cord, such as the fourth ventricle of the brain (in the back, inside) and within the central canal of the spinal cord. Occasionally they grow just beneath the surface of the lateral ventricles (called "subependymoma").

Although these tumors are capable of malignant behavior, they are almost always benign. Surgical removal often leads to a cure, especially when the tumor arises right from the surface to the fluid spaces of the brain and grows into the ventricles, an "exophytic" growth, allowing for complete removal.

If you or yours has this tumor, be confident that your future holds promise.

CEREBELLAR ASTROCYTOMA

This tumor is quite cureable. The small cyst version is one that, when surgically removed, is gone for good. Forget about all the fears, just thank God that your child will do well. Even if the MRI or CAT scan shows a large cyst with surrounding tumor or a tumor "nodule" in the wall of the cyst, the surgery can go well.

When the tumor is the more aggressive type, the outcome deplends on the grade/size of the tumor.

TREATMENT OF CHILDHOOD BRAIN TUMORS

The treatment of these tumors usually include a combination of approaches, each tailored to deal with the problems that children are presented with:

1. Surgery:- In some cases the definitive treatment is surgery. In most, however, surgery serves as a temporizing measure that will keep a child out of trouble for long enough to get through definitive therapy that will hopefully eliminate of the tumor. Brain surgery is usually the easiest part of a child's treatment.
2. Shunting:- Quite often (e.g. medulloblastomas) childhood tumors present the blockage of the fluid spaces of the brain, (obstructive hydrocephalus). In Shunting, a thin silastic tube (the shunt) is placed into the fluid spaces of the brain, passed under the skin into the child's tummy where the fluid is absorbed.
3. Chemotherapy:- Unfortunately, chemotherapy is the hard part of brain tumor treatment. It is only required for the more aggressive tumors. As a rule, chemotherapy should be even more aggressive than the tumor itself. The trials which are imposed both on the child and parents are legion. Bravery and an unremitting attitude of hope are required by all involved.
4. Radiation:- Because the developing brain of a child is so very sensitive to radiation therapy, it is deliberately limited. The irony of effective radiation therapy is that when it works well, the brain damage it causes might exceed that done by the original tumor. More often than not, your doctor will recommend that if any radiation is to be given, it should be held off until the child has grown older and the brain has sufficiently matured.

The Good News is that REAL HOPE exists. Long term results are becoming more and more common, some children surviving fifty years and beyond. This has become especially true with the wonderful improvements in chemotherapy and surgical techniques. The promise of cures for difficult tumors is becoming a reality to more families than ever before.