Tumors involving the infratentorial region are more common than those involving the supratentorial region in children. They can arise either in the brainstem or the cerebellum. The clinical presentation differs based on the region in which the tumor arises.

Tumors involving the cerebellum often present in a subtle manner. As the tumor grows, it blocks the flow of cerebral spinal fluid (CSF) through the fourth ventricle. As the brain parenchyma, CSF, and blood are enclosed by a rigid skull and the skull cannot expand to accompany the increase in CSF, increased intracranial pressure (ICP) occurs. The initial presentation of raised ICP often consists of morning headaches occurring upon awakening [3]. The headache may or may not initially be associated with effortless, projectile vomiting. When the child has been standing or sitting for a few minutes, the symptoms can significantly decrease as a result of reestablishment of CSF flow due to the effect of gravity and change in position of the tumor mass. Though headaches are common in children, early morning headache with vomiting, an intense headache or one that awakens the child from sleep, a headache that worsens with cough, defecation or Valsalva maneuver, and the presence of papilledema on fundoscopy should alert the physician of possibility of a cerebellar tumor [1, 4, 5].

Increased ICP can also be associated with non-localizing palsy of the sixth (abducens) cranial nerve, impaired light reflex, and a head tilt. The abducens nerve innervates the ipsilateral lateral rectus muscle which abducts the eye. It has the longest intracranial course of all the cranial nerves and is usually the first nerve affected by elevated ICP. Symptoms include binocular horizontal diplopia which is worst at distance and esotropia. In order to minimize diplopia, children may tilt their head to reduce double vision. A more extreme presentation of rapidly rising ICP consisting of a decreased level of consciousness or Cushing’s triad (elevated blood pressure, bradycardia, and irregular respiration) is uncommon. In these situations, a rapidly growing tumor of the midline brainstem or posterior fossa should be suspected and immediate intervention is critical [3].

Cerebellar signs such as clumsiness in movements, speech, dysmetria, and nystagmus often occur late and are more common in tumors that involve the cerebellar hemispheres. Truncal ataxia, when present, suggests that the cerebellar vermis is involved.

Brainstem tumors present in a different manner. Increased intracranial pressure, if it occurs at all, occurs late. As the brainstem contains the cranial nerve nuclei and the motor long tracts, cranial nerve palsies with or without motor difficulties are common at presentation. The motor difficulties can be hemiparesis, monoparesis, tetraparesis, or quadriparesis.

Cranial neuropathies are another common presentation of brainstem tumors. Abnormalities of extraocular movements are common findings. Diplopia and head tilt suggest impairment of cranial nerve IV or cranial nerve VI. Involvement of other cranial nerves may cause facial weakness, hearing loss, abnormal corneal or gag reflex, or difficulties with swallowing [1].

Supratentorial tumors include tumors of the cerebellar hemispheres, basal ganglia, and thalamus and hypothalamus regions (gliomas); ventricular neoplasms (choroid plexus papillomas and ependymomas); parasellar tumors (craniopharyngiomas, optic pathway gliomas, germinomas); and other less common tumors such as pinealomas. In addition to raised intracranial pressure, supratentorial tumors often present with localizing symptoms and signs. These symptoms are generally related to the location of the tumor and can vary from indolent progression to an acute presentation based on the tumor growth rate.

A focal seizure can be a common presenting symptom of a tumor involving the cerebral hemisphere. As a rule, seizures do not occur in patients with infratentorial tumors [3]. Depending on the site of the tumor, the pattern of seizure may be different and occasionally identification of patient’s symptoms based on seizure is challenging, especially in infants and toddlers. The seizures are often resistant to standard antiepileptic drug therapy.