Central Nervous System tumors

Pediatric CNS tumors are the second most common tumors after leukemia in this age group and the most common cause of mortality and morbidity.

Children with CNS tumors usually have a longer time between the onset of symptoms and diagnosis compared to other tumors. There are many reasons for this but the non-specificity of the presenting symptoms and signs and the lack of awareness about CNS tumors among healthcare providers and the community seem to be the major contributing factors.

What are the symptoms of CNS tumor?

The symptoms and signs attributed to CNS tumors vary according to the age of the child. Younger children may have persistent/recurrent vomiting, walking or balance problems, abnormal eye movements/visual loss, behavioral changes (particularly lethargy), head tilt, increasing head circumference, and convulsions or altered level of consciousness. Older children may have, in addition to the above, recurrent or persistent headaches, blurry or double vision, back pain/curved back, delayed or arrested puberty, and polyuria or polydipsia.

A proper clinical assessment with a detailed history and physical examination by the healthcare provider can determine if a CNS imaging, whether a CT and/or an MRI scan, is required.

How are CNS tumors treated?

Treatment of a CNS tumor depends on the patient’s clinical status, the tumor location, the radiological tumor characteristics, the presence or absence of metastasis, and the histological diagnosis (if a tumor biopsy is performed). Treatment may include surgical resection, chemotherapy and/or radiotherapy, or any combination of these modalities based on the tumor diagnosis. In some occasions, new medications targeted to specific tumor mutations may be used. In all cases, the treatment plan needs to be discussed among a multidisciplinary team consisting at minimum of a pediatric oncologist, a neurosurgeon, a radiologist, a radiation oncologist and a pathologist - preferably before the surgical intervention (in non-urgent cases) and definitely after surgery due to the expected long-term morbidities associated with a diagnosis of a CNS tumor.

Prognosis of CNS tumors in children depends mainly upon the histological diagnosis and the tumor location, which determines the extent of the surgical resection. Unfortunately, there have been no major improvements in the outcomes of pediatric CNS tumors compared to leukemia for the past few decades. The overall prognosis is in the range of 60-70%, but some tumor types may have a dismal outcome of <20%. Survivors of CNS tumors may have long term morbidities related to the tumor or its treatment. These may include physical morbidities, neurocognitive deficits, endocrine deficits, visual loss, or hearing deficits. These challenges often negatively affect the child’s academic performance and his or her social integration in the community. For these reasons, multidisciplinary team discussions are important to help minimize or treat these morbidities.