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Yibian
 Shen Yaozi 
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diseasePediatric Intracranial Tumors
aliasIntracranial Tumor
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bubble_chart Overview

Pediatric intracranial tumors rank second in incidence among childhood tumors, only after leukemia. They can occur at any age, with the highest incidence between 5 to 8 years old. In children, infratentorial tumors are more common, often gliomas, which have a poorer prognosis. In recent years, advancements in surgical and anesthetic techniques, improved postoperative care, and the application of comprehensive treatment measures have contributed to a decline in surgical mortality rates.

bubble_chart Auxiliary Examination

  1. Lumbar puncture: Can measure intracranial pressure. In infants and young children, crying often makes pressure measurements less reliable, while results in older children are more valuable. Tumor cells may be found in the cerebrospinal fluid for certain brain tumors (e.g., medulloblastoma, ependymoma, etc.), which is highly significant for definitive diagnosis. However, if the child already exhibits obvious symptoms of increased intracranial pressure or is at risk of brain herniation, lumbar puncture should be contraindicated.
  2. Skull X-ray: May suggest the possibility of intracranial space-occupying lesions but cannot confirm a diagnosis. In cases of elevated intracranial pressure, skull X-rays may show widened cranial sutures and increased convolutional markings. For pituitary region lesions, the X-ray may reveal an enlarged sella turcica and decalcification or destruction of the anterior and posterior clinoid processes. A normal X-ray does not rule out a brain tumor.
  3. Electroencephalogram (EEG) and brain mapping: For tumors located in the cerebral hemispheres, most cases show focal delta waves on EEG, and brain mapping may reveal a focal high-power delta wave area at the tumor site. EEG lacks localizing value for posterior fossa tumors and ventricular system tumors, but background activity slows.
  4. CT scan: Approximately 90% of brain tumors can be diagnosed via CT, and the method is straightforward. For suspected cases, intravenous contrast enhancement can improve diagnostic accuracy. However, infratentorial tumors are often obscured by artifacts due to the posterior fossa being surrounded by bone and air-filled sinuses.
  5. Magnetic Resonance Imaging (MRI): MRI allows coronal, sagittal, and axial cross-sectional scans, providing highly accurate tumor localization and clear differentiation between gray and white matter. For tumors in the sellar region, brainstem, or posterior fossa, the absence of bone artifacts enables detection of even small tumors.

bubble_chart Diagnosis

(1) Common Symptoms

  1. Vomiting is the most common symptom of intracranial tumors in children, especially in infants and young children. Sometimes it is the only early symptom. Vomiting is often not accompanied by nausea and is unrelated to diet, mostly being projectile. In the early stages of the disease, vomiting often occurs in the morning.
  2. Headache is mostly caused by increased intracranial pressure, while in a few cases, it may result from direct stimulation of the dura mater by the tumor, leading to localized headache. Headache can be intermittent or continuous. Severe headache may be accompanied by vomiting. Young children often cannot express their headache and may only show paroxysmal crying, restlessness, or grabbing or hitting their heads.
  3. Visual impairment is a common symptom of intracranial tumors in children but is often overlooked. Sometimes, it is not noticed by parents until the child develops bilateral blindness or near-blindness.
  4. Gait instability and ataxia are common due to the higher incidence of infratentorial tumors in children. When the cerebellar vermis is affected, it often manifests as unsteady gait and frequent falls. If the tumor involves the cerebellar hemisphere, it may present with ataxia such as unsteady pointing and tremors in the ipsilateral limb.
  5. Polydipsia and polyuria are often caused by tumors in the sellar region compressing the pituitary gland and hypothalamus, affecting the secretion of antidiuretic hormone. Tumors in the anterior part of the third ventricle can also cause these symptoms.
  6. Mental and emotional abnormalities may occur in some children due to increased intracranial pressure, manifesting as apathy, lack of strength, drowsiness, disinterest in play, dull or irritable responses, and dysphoria.
  7. Seizures occur in about 20-50% of cases with cerebral hemisphere tumors, often presenting as tonic-clonic seizures, though focal seizures may also be seen.
  8. Fever is not uncommon during the course of intracranial tumors in children. It may be related to the malignant nature of the tumor, hemorrhage, necrosis, or tumor cells shedding into the cerebrospinal fluid, or it may be associated with thermoregulation. Fever should not be attributed solely to intracranial inflammation.

(2) Signs

  1. Head enlargement is more common in infants and young children, caused by the separation of cranial sutures due to increased intracranial pressure. Percussion may reveal a cracked pot sound (positive Macewen's sign).
  2. Papilledema is one of the important signs of intracranial tumors in children, with a positive rate of about 70-80%. Papilledema is mostly bilateral and symmetrical, though sometimes the degree of edema may differ between the two sides.
  3. Neck stiffness is more common in children with posterior fossa tumors, possibly due to compression or stimulation of the upper cervical nerve roots by the tumor or cerebellar tonsils.
  4. Forced head posture occurs more frequently in children than in adults and is a protective reflex. The child often adopts a specific posture to maintain a special head position, ensuring the smooth circulation of cerebrospinal fluid. This is mostly seen in posterior fossa tumors or tumors in the lateral and third ventricles.
  5. Nystagmus is more common in cerebellar tumors, often presenting as coarse horizontal nystagmus, which becomes more pronounced when gazing toward the side of the tumor.
  6. Pyramidal signs may be positive in supratentorial tumors, especially cerebral hemisphere tumors, with bilateral hyperreflexia and a positive Babinski sign. In cerebellar tumors, positive pyramidal signs are often seen only in the advanced stage.

bubble_chart Treatment Measures

  1. Surgical treatment: Includes radical surgery and palliative surgery. The former aims to remove the tumor as thoroughly as possible, while the latter is intended to relieve symptoms, alleviate the tumor's compression on major functional structures, reduce intracranial pressure, and resolve cerebrospinal fluid circulation obstruction. Improving postoperative patient monitoring is a crucial measure to reduce surgical mortality and improve prognosis.
  2. Radiation therapy: Primarily targets highly malignant tumors, those that cannot be completely resected surgically, or recurrent tumors post-surgery. Among pediatric intracranial tumors, medulloblastomas and other types of gliomas are the most sensitive to radiation therapy. Additionally, germ cell tumors, pineal cell tumors, and pituitary adenomas are also relatively sensitive.
  3. Chemotherapy: Can serve as an adjunctive treatment for intracranial tumors, mainly targeting residual tumor cells after surgery rather than the main tumor mass. It is often administered concurrently with radiation therapy following surgical resection.
  4. Immunotherapy: Aims to inhibit tumor growth or eliminate tumor cells by leveraging the body's own immune defense system. It includes two major categories: specific and non-specific treatments. The former involves tumor-specific antigens, immune sera, or immunocompetent cells, which stimulate the body's own disease-fighting capabilities through external stimuli. The latter is achieved using agents such as BCG, Corynebacterium parvum, interferons, and inducers.

bubble_chart Differentiation

  1. Nervous system infections: Brain tumors are often misdiagnosed as tuberculous meningitis, purulent meningitis, or viral encephalitis. The reasons for misdiagnosis include:
    1. Since malignant tumors are more common in children, necrosis or hemorrhage of tumor tissue can cause fever, often leading to misdiagnosis as a nervous system infection;
    2. Some brain tumor cells shed into the cerebrospinal fluid, making them easily confused with white blood cells under a microscope;
    3. Posterior fossa tumors are more common in children. When the tumor protrudes into the spinal canal through the foramen magnum or the cerebellar tonsillar hernia stimulates the upper cervical nerve roots, the child's protective reflex causes the head to tilt backward, presenting as neck stiffness during examination, which can be mistaken for meningeal irritation signs.
  2. Gastrointestinal disorders: Due to the high incidence of vomiting in brain tumors, it often remains the primary symptom for an extended period in the early stages of the disease. Some children with posterior fossa tumors may also experience abdominal pain in addition to headaches, further increasing the likelihood of misdiagnosis as gastrointestinal disorders.
  3. Congenital hydrocephalus: In infants and young children, intracranial tumors often present with increased head circumference, elevated tension in the anterior fontanelle, and distended scalp veins due to elevated intracranial pressure, closely resembling hydrocephalus. However, congenital hydrocephalus typically manifests early, with gradual head enlargement soon after birth; the eyes often exhibit a "setting sun" sign; optic disc edema is rare; vomiting is uncommon; and neurological focal signs are seldom observed. These features help differentiate it from intracranial tumors.

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