symptoms and treatment of Spinal cord tumors in children

Dr. Moradi, a neurologist

Spinal cord tumors in children are rare, but they do happen. In fact, tumors of the nervous system are the most common type of solid tumors (those not originating in the blood or bone marrow) found in children. Spinal cord tumors can be life threatening if left untreated. The following information will give you a better understanding of this potentially serious medical condition.

What are Spinal Cord Tumors? 

Spinal tumors, also called neoplasms, are abnormal growths of tissue found inside the spinal column. They can be benign (non-cancerous) or malignant (cancerous). Tumors that originate in the spine are called primary tumors. Whether cancerous or non-cancerous, tumors in the spine can cause serious health problems as they grow and place pressure on the spinal cord. Immediate treatment is usually required.

Symptoms of Spinal Cord Tumors

Symptoms of spinal tumors generally develop slowly and worsen over time. The main symptom is chronic back pain. Other symptoms may include one or more of the following:

  • Sciatica
    • Numbness, weakness
    • Partial paralysis
    • Spinal deformity
    • Difficulty with bladder control
    • Fever

While some of these symptoms may be difficult to recognize in very young children and infants, regular checkups with a pediatrician can help detect many potential health problems, including tumors.

How is a spinal cord tumor diagnosed in children?

The first step in diagnosing a spinal cord tumor includes a discussion of the child's health history and a thorough physical examination. If a spinal cord tumor is suspected, the child will need to undergo some diagnostic tests to rule out other possible health problems. These tests may include:

  • Laboratory tests of the child's blood and spinal fluid for the presence of tumor cells
  • X-ray of the spine - to detect the presence of scoliosis or bone erosion from the tumor
  • MRI - for a more detailed look at the structures of the spinal cord and/or location of the tumor
  • CT scan  to see if there are other areas of the body affected

What Are the Risk Factors for Brain and Spinal Cord Tumors in Children?

A risk factor is anything that affects a person’s chance of getting a disease such as a brain or spinal cord tumor. Different types of cancer have different risk factors.

Lifestyle-related risk factors such as diet, body weight, physical activity, and tobacco use play a major role in many adult cancers. But these factors usually take many years to influence cancer risk, and they are not thought to play much of a role in childhood cancers, including brain tumors.

Very few risk factors have been found for spinal cord and brain tumors. There is no clear cause for most of these tumors.

Radiation exposure

The only well-established environmental risk factor for brain tumors is radiation exposure to the head, which most often comes from the treatment of other conditions.

For example, before the risks of radiation were well known (more than 50 years ago), children with ringworm of the scalp (a fungal infection) often received low-dose radiation therapy. This was later found to increase their risk of brain tumors as they got older.

Today, most radiation-induced brain tumors are caused by radiation given to the head to treat other cancers, such as leukemia. These brain tumors usually develop around 10 to 15 years after the radiation.

Radiation-induced tumors are still fairly rare, but because of the increased risk (as well as the other side effects), radiation therapy is only given to the head after carefully weighing the possible benefits and risks. For most patients with cancer in or near the brain, the benefits of getting radiation therapy as part of their treatment far outweigh the small risk of developing a brain tumor years later.

The possible risk from fetal or childhood exposure to imaging tests that use radiation, such as x-rays or CT scans, is not known for sure. These tests use much lower levels of radiation than those used in radiation treatments, so if there is any increase in risk, it is likely to be very small. But to be safe, most doctors recommend that pregnant women and children not get these tests unless they are absolutely needed.

Inherited and genetic conditions

In rare cases (less than 5% of brain tumors), children have inherited abnormal genes from a parent that put them at increased risk for certain types of brain tumors. In other cases, these abnormal genes are not inherited but occur as a result of changes (mutations) in the gene before birth.

People with inherited tumor syndromes often have many tumors that start when they are young. Some of the more well-known syndromes include:

Neurofibromatosis type 1 (von Recklinghausen disease) 

This is the most common syndrome linked to brain or spinal cord tumors. It is often inherited from a parent, but it can also start in some children whose parents don’t have it. Children with this syndrome may have optic gliomas or other gliomas of the brain or spinal cord, or neurofibromas(benign tumors of peripheral nerves). Changes in the NF1  gene cause this disorder.

Neurofibromatosis type 2

Less common than von Recklinghausen disease, this condition can also either be inherited or may start in children without a family history. It is associated with cranial or spinal nerve schwannomas , especially vestibular schwannomas (acoustic neuromas), which almost always occur on both sides of the head. It is also linked to an increased risk of meningiomas , as well as spinal cord gliomas or ependymomas . Changes in the NF2 gene are responsible for neurofibromatosis type 2.

Tuberous sclerosis

Children with this condition may develop subependymal giant cell astrocytomas (SEGAs), as well as other benign tumors of the brain, skin, heart, kidneys, or other organs. This condition is caused by changes in either the TSC1 or the TSC2 gene.

Von Hippel-Lindau disease

Children with this disease tend to develop blood vessel tumors (hemangioblastomas) of the cerebellum, spinal cord, or retina, as well as tumors in the kidney, pancreas, and some other parts of the body. It is caused by changes in the VHL gene.

Other syndromes

Other inherited conditions linked with increased risks of certain types of brain and spinal cord tumors include:

  • Gorlin syndrome (basal cell nevus syndrome)
  • Turcot syndrome
  • Cowden syndrome
  • Hereditary retinoblastoma
  • Rubinstein-Taybi syndrome

Some families may have genetic disorders that are not well recognized or that could even be unique to a particular family.

treatment

Immediate treatment of a spinal cord tumor is the key to a successful outcome. Most cases of spinal cord tumors are treated surgically. As stated above, even non-cancerous tumors can continue to grow and place pressure on the spinal cord and block the blood vessels that nourish spinal cord cells. This can seriously affect the child's ability to function and can even cause total and permanent paralysis.

Surgery is performed to remove or reduce the size of the tumor and alleviate the pressure on the spinal column caused by the tumor. Once the surgery has been performed, a pathologist examines tumor tissue to determine the exact type of tumor. This is called tumor classification and is based on the type of cells from which the tumor originates. After the tumor is classified, it is then given a number to indicate the speed at which it is growing. This is called tumor grading. A higher number is given to fast-growing tumors and less aggressive tumors are given lower numbers.

In addition to surgery, other parts of the treatment plan may include:

  • Radiation therapy - x-ray beams targeted to the tumor site to destroy tumor cells
  • Chemotherapy - used for cancerous tumors to destroy cancer cells
  • Physical therapy - to help restore body functions and strength impaired or lost before surgery

Rehabilitation

Depending on the size and location of the tumor, most children tolerate this type of surgery quite well and are often released from the hospital within a few days of the procedure. Once home, the child may need to be given pain medications until the incision is completely healed.

As soon as the child is able, he or she should be encouraged to return to daily activities like school or play. Exercise can be done in moderation. The child should also get plenty of rest and be given a well-balanced diet.

Follow-up with the child's surgeon to monitor the recovery process is important. Regular check-ups with the child's pediatrician should also continue.