Intracranial Tumors

Symptoms and signs are related to the location, size, and growth rate of the tumor:

1. Slow-growing tumors produce massive shifts of normal structures and may become quite large by the time they first become symptomatic.

Table 17-2. Cytogenetic loci implicated in malignant brain tumors in children

Tumor Chromosome Disorder

PNET/MB 5q21 Turcot syndrome

9q22.3 NBCCS

17p13.3 17q

Astrocytoma, grades III-IV 7p12

17p13.1 Li-Fraumeni syndrome

17q11.2 Neurofibromatosis-1

Subependymal giant cell tumor 9q34, 16p13 Tuberous sclerosis

Meningioma 22q

22q12 Neurofibromatosis-2

ATT/Rhabdoid tumor 22q11.2

Abbreviations: PNET/MB, prototype primitive neuroectodermal tumor, medulloblastoma; ATT/RT, atypical teratoid rhabdoid; NBCCS, nevoid basal cell carcinoma syndrome.

Adapted from Biegel JA. Genetics of pediatric central nervous system tumors. J Pediatr Hematol Oncol 1997; 19:492-501, with permission.

2. Rapidly growing tumors produce symptoms early and present when they are relatively small.

The most common presenting signs and symptoms of an intracranial neoplasm are increased intracranial pressure (ICP) and focal neurologic deficits. If symptoms and signs of increased ICP precede the onset of localized neurologic dysfunction, a tumor of the ventricles or deep midline structures is most likely. If localizing signs (seizures, ataxia, visual field defects, cranial neuropathies, or corticospinal tract dysfunction) are predominant in the absence of increased ICP, it is more probable that the tumor originates in parenchymal structures (cerebral hemispheres, brain stem, or cerebellum).

General signs and symptoms of intracranial tumors:

1. Headache. In young children headache can present as irritability. Often worse in the morning, improving throughout the day.

2. Vomiting (often early morning).

3. Disturbances of gait and balance.

4. Cranial nerve abnormalities.

5. Impaired vision:

a. Diplopia (6th nerve palsy). In young children diplopia may present as frequent blinking or intermittent strabismus.

b. Papilledema from increased ICP may present as intermittent blurred vision.

c. Parinaud syndrome (failure of upward gaze and setting-sun sign, large pupils and decreased constriction to light).

6. Mental disturbances: somnolence, irritability, personality or behavioral change, or change in school performance.

7. Seizures, usually focal.

8. Endocrine abnormalities: Midline supratentorial tumors may cause endocrine abnormalities due to effects on the hypothalamus or pituitary and visual field disturbances due to optic pathway involvement.

9. Cranial enlargement (characteristic of increased ICP in infants).

10. Diencephalic syndrome can be seen in patients aged 6 months to 3 years with brain tumors who present with sudden failure to thrive and emaciation. The syndrome is caused by a hypothalamic tumor in the anterior portion of the hypothalamus or the anterior floor of the third ventricle.

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