Upratentorial Primitive Neuroectodermal Tumors, Childhood

Supratentorial primitive neuroectodermal tumors are fast-growing tumors that form in brain cells in the cerebrum. The cerebrum is at the top of the head and is the largest part of the brain. The cerebrum controls thinking, learning, problem-solving, emotions, speech, reading, writing, and voluntary movement. Childhood supratentorial primitive neuroectodermal tumors are also called cerebral neuroblastomas or cerebral medulloblastomas.

Supratentorial primitive neuroectodermal tumors

 

Although cancer is rare in children, brain tumors are the most common type of childhood cancer other than leukemia and lymphoma. This summary refers to the treatment of primary brain tumors (tumors that begin in the brain). Treatment of metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary. Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. (Refer to the PDQ treatment summary on Adult Brain Tumors for more information.)

The cause of most childhood brain tumors is unknown.
The symptoms of childhood supratentorial primitive neuroectodermal tumors and pineoblastoma vary and often depend on the child’s age, where the tumor is located, and the size of the tumor. These symptoms may be caused by a supratentorial primitive neuroectodermal tumor, a pineoblastoma, or by other conditions. A doctor should be consulted if any of the following problems occur:

• Weakness or change in sensation on one side of the body.


• Morning headache or headache that goes away after vomiting.


• Nausea and vomiting.


• Seizures.


• Unusual sleepiness or change in energy level.


• Change in personality or behavior.


• Unexplained weight loss or weight gain.

Besides the normal scans that will be performed such as X-ray, CT scans and MRI's there will also be laboratory testing.

If doctors think your child may have a CNS embryonal tumor, a biopsy may be done to remove a sample of tissue. For brain tumors, the biopsy is done by removing part of the skull and using a needle to remove a sample of tissue. Sometimes, a computer-guided needle is used to remove a sample of tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery.
The following tests may be done on the sample of tissue that is removed:

• Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.


• Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.


• Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.


• Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.

 TREATMENT
The standard treatment is surgery, radiation, chemotherapy and stem cell transplant. Below is a video of mother dealing with the trauma of her daughter who has this brain tumor.  Cancer is traumatic for anyone and their family.  Support of such individuals is very important.

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