Pediatric CNS Tumors - Causes, Symptoms, and Treatment.

Introduction

Pediatric CNS (central nervous system) tumors are common solid cancers after leukemia. It can occur soon after the child is born or can occur with birth. Certain tumors can be detected during the second and third trimesters, and the required treatment is started after the birth. Emergency care is required in case of pediatric CNS tumors. Early detection and treatment play a major role in saving the life of the child. The diagnosis, along with the treatment plan with the subsequent risks, is discussed with the family members and proceeded.

What Are Pediatric CNS Tumors?

Pediatric CNS tumors are tumors of the central nervous system occurring in children and also in infants. The central nervous system, which includes the brain and the associated nerves, plays a major role in the well-being of the child. Any tumor, mass, lump, or cancer occurring in the child can turn out to be fatal.

What Are the Various Types of Pediatric CNS Tumors?

Pediatric CNS tumors can be classified as the following -

Based on the Location:

• Supratentorial Tumors - The tentorium is a part of the brain that helps in protecting the brain against pressure. It is the most common tumor in children of age less than three years.

• Infratentorial Tumors - Tumors present below the tentorium are the infratentorial tumors

Based on the Age:

• Congenital Brain Tumors - Tumors diagnosed within two months of life.

• Tumors of Infancy - Tumors detected in less than two years after birth.

• Tumors of Older Children -Tumors detected after two years of birth.

• Germ Cell Tumor - Germ cell tumor, known as teratoma, is the most common tumor in childhood, equally occurring in girls and boys.

• Astrocytic tumors - Glioblastoma multiformae.

• Embryogenic tumors - Medulloblastoma.

• Tumors of choroid plexus - Choroid plexus papilloma.

Certain other pediatric tumors include:

1. Tumors of peripheral nerves.

2. Neurofibroma.

3. Schwanomma.

4. Meningeal tumors.

5. Meningioma.

6. Atypical meningioma.

7. Elastic meningioma.

8. Desmoplastic infantile tumors.

What Are the Causes of Pediatric CNS Tumors?

The exact cause of CNS tumors is still being researched. It is also believed that environmental and genetic factors can cause pediatric CNS tumors. The major role is also played by family history. High-dose radiation for treating leukemia in children has also been a cause of the development of CNS malignancies in children.

What Are the Symptoms Associated With Pediatric CNS Tumors?

The following are the clinical findings in pediatric CNS tumors -

• Obstruction in the cerebrospinal fluid drainage leads to increased intracranial pressure (pressure inside the head).

• Hydrocephalus (abnormal accumulation of fluid in the deeper parts of the brain).

• Nausea and vomiting.

• Headache.

• Personality changes (loss of balance, blurred speech).

• Macrocephaly (increase in the size of the head of an infant).

• Visual impairment (abnormal vision).

• Seizures (sudden uncontrolled bursts of movements).

• Ophthalmoplegia (weakness in the muscles of the eyes).

• Scoliosis (abnormal curvature of the spine towards sideways).

• Papilledema (swelling of the optic nerve).

• Endocrine deficits (defects related to hormonal changes).

• Memory problems.

What Are the Diagnostic Methods for Pediatric CNS Tumors?

CNS tumors in childhood can be diagnosed by the following methods -

Congenital tumors can be diagnosed on an ultrasound during the third trimester itself as an intracranial mass (mass in the brain).

• MRI (Magnetic Resonance Imaging) - The presence of varying signal intensities denotes the occurrence of fatty lesions that are pathologic.

• CT (Computed Tomography) - Mixed-density lesions are seen along with calcifications.

• Sonography - In infants, sonography is performed to detect the presence of cerebral edema or hemorrhage.

• Spinal MRI - Spinal MRI is performed to detect the presence of any metastasis before performing the surgery.

• Amino Acid PET (Positron Emission Tomography) -Amino acid PET scan is used to assess the progress of the treatment.

• Histopathological Evidence - To combat the imaging artifacts, the laboratory examines the tissues.

• Bone scan - A particular dye is injected into the body and absorbed by the bones, and the x-ray is taken.

• Angiogram - Visualizes all the blood vessels in the brain to detect the presence of any kind of tumor in the brain.

What Are the Treatment Strategies for Pediatric CNS Tumors?

• Emergency care is initiated soon after the detection of the tumor. The patient is not made to lie down flat, rotation of the head should not be done, and Dexamethasone is administered. CSF (cerebrospinal fluid) drainage is done until the surgery is performed. Surgical resection is performed when the risk of the surgery is acceptable.

• In the case of medullary compression, neurosurgery is indicated.

• Laminectomy and fixation are included in the surgical procedure.

• Complete removal of the tumor by microsurgery is the ultimate goal of the surgery. Low-grade gliomas are ultimately treated only by surgery.

• Management of pediatric CNS tumors is a multidisciplinary approach. Counseling and support to the family members make a major difference in the treatment outcome. Genetic testing is also done in parents to avoid risks of occurrence of other malignancies and future family planning.

• Surgical resection of the entire mass

• Cranio-spinal radiotherapy is used in patients less than eight years old are treated by chemotherapy mostly and, in rare cases, through radiation.

• Adjuvant therapy is indicated in patients where there are decreasing signs of vision.

• Chemotherapy involves administering steroids to reduce the swelling in the brain.

• Supportive care is also given for the side effects of treatment or the tumor.

What Is the Prognosis for Pediatric CNS Tumors?

The prognosis of the tumor depends on the age of occurrence of the tumor, the intensity of the tumor, its type, and the extent of resection. It is to be remembered that among all the cancers occurring in children, brain tumors are the most common reason for death in them. It can be stated that the prognosis of pediatric CNS tumors is poor.

What Are the Complications Associated With Pediatric CNS Tumors?

Complications of pediatric CNS tumors can occur due to the presence of a tumor or due to the treatment.

• Neurological deficits.

• Severe pain.

• Seizures.

• Recurrence.

Conclusion

Detecting the condition at an early stage and immediate action toward the tumors by a pediatric neurosurgeon and a pediatric oncologist is essential. The best care for the patient is delivered by a team involving oncologists, pathologists, nurses, rehabilitation members, and social workers. As there are complications associated with pediatric tumors, early detection of the condition can help the patient recover faster, which provides a better outcome.