Types of tumors

Brain stem tumors

  • 10– 20% of tumors
  • The most vicious and malignant tumors in childhood
  • 80% of these tumors are malignant astrocytomas (glioblastoma) and grow rapidly, particularly those occurring in the part of the brain known as the pons

Symptoms

  • One eye turning inward, facial droop, difficulty to swallow
  • Weakness of extremities
  • Ataxia (poor muscular coodination)
  • One-third of patients have hydrocephalus

Unfortunately, a great majority of tumors in the pons (mid-portion of the brain stem) are not surgically resectable. Total resection may be attempted in those occurring in other parts of the brain stem.

Treatment

  • Radiation and chemotherapy together for pontine tumor, but prognosis is extremely poor (two-year survival: 8%)
  • Mid-brain or medulla oblongata tumors may be resected without further therapy

Cerebellar astrocytomas

  • One of the most benign and favorable tumors among pediatric brain tumors
  • Hydrocephalus in 90%

Possible symptoms

  • Morning headaches
  • Nausea
  • Progressive unsteadiness of gait, (if patient has hydrocephalus)
  • Head tilt and pain in the back of the head (if patient does not have hydrocephalus)

Treatment

  • Surgical resection. Once tumor is completely removed, approximately 95% of patients are cured without further treatment

Cerebral PNET / glioblastoma

  • One of the most primitive and malignant tumors among CNS tumors in pediatrics
  • Tumors tend to be large and have many blood vessels

Symptoms

  • Morning headaches
  • Nausea
  • Progressive unsteadiness of gait
  • Seizures
  • Visual disturbances

Treatment

  • Attempt resection, then radiation and chemotherapy—even if total resection
  • Radiation may be required to the spine because of the tumor's tendency to spread

Choroid plexus papilloma

  • Usually benign
  • 4% of brain tumors
  • Formed within the ventricle
  • two-thirds of patients are under two years of age

Symptoms

  • Headaches and emesis
  • Lethargy (related to hydrocephalus)

Treatment

  • Surgical resection
  • Seizures or hydrocephalus may be a problem post-operatively

Craniopharyngioma

  • 5–13 % of tumors
  • Arises in embryonically misplaced cells in the base of the brain
  • A large number are surgically resectable, however, endocrine function is often disturbed post-operatively

The most common endocrine problems are lack of growth hormone production, adrenocordicoidal hormone, sex and thyroid hormone, and diabetes insipidus (too much urine output and extreme thirst).

Symptoms

  • Visual disturbance
  • Possible hydrocephalus

Treatment

  • Surgical resection
  • Possible radiation to any residual tumor
  • Frequent endocrine abnormalities following radical tumor resection, needing hormone replacement therapy

Ependymomas

  • 10% of brain tumors
  • May invade the brainstem
  • Histologically benign, but behaves malignant
  • Recurrence rates are much higher than even medulloblastomas
  • Hydrocephalus in 85%

Symptoms

  • Morning headaches
  • Nausea
  • Progressive unsteadiness of gait

Treatment

  • Surgical resection followed by radiation if only a partial resection was possible
  • If totally resected, no further therapy with close surveillance MRIs
  • Extent of surgical resection is the major factor for outcome
  • Recurrent tumor is difficult to treat: treatment is resection followed by chemotherapy
  • Currently, the five-year survival rate is as low as 40% for posterior fossa ependymoma tumors

Hypothalamic Tumors

  • Most are benign astrocytomas and are slow growing.

Symptoms

  • Endocrine problems such as precocious puberty and diencephalon syndrome (loss of weight, poor subcutaneous fat development despite good appetite and increasing height).

Treatment

  • Surgical removal of part of the tumor is possible but not a total resection. First choice is chemotherapy, then radiation if no response to chemotherapy.

Medulloblastomas

  • 15–20% of tumors
  • Often presents in the cerebellum and fourth ventricle—resulting in hydrocephalus
  • Is malignant and grows rapidly
  • Tends to spread through CSF circulation

Although malignant, this is a pediatric brain tumor in which marked improvement of survival and quality of life have been observed. Decades ago the five year survival rate was approximately 20%. The five-year survival rate now is 70–80%.

Survival is influenced by multiple factors. Other than medical and biological factors, the institution (a university-affiliated hospital like Children's Memorial Hospital vs. a community hospital) may make a large difference in patient's prognosis.

Symptoms

  • Morning headaches
  • Nausea
  • Progressive unsteadiness of gait

Treatment

  • Surgical removal followed by radiation and chemotherapy.

Optic chiasm and hypothalamic gliomas

  • A majority of these tumors are benign astrocytomas and are slow growing 30% of optic gliomas are associated with neurofibromatosis
  • More aggressive if they occur during the first two years of life

Symptoms

  • Visual acuity changes
  • Nystagmus (involuntary rapid and rhythmic movement of the eyeball)
  • Failure to thrive
  • Hydrocephalus

Treatment

  • Because they are unresectable, the first choice of treatment is chemotherapy. Radiation is used if tumor progresses. Surgical resection is rarely recommended.

Pineal Region Tumor

  • Malignant in 80% of cases
  • 3–8% of tumors
  • Almost invariably present with hydrocephalus
  • Tumors may secrete specific tumor markers, named Beta HCG Alpha fetoprotein

Treatment

  • Some tumors may be resected, but it is difficult
  • Surgery/radiation/chemotherapy are needed for most malignant pineal tumors
  • Malignant germ cell tumors respond to chemotherapy so that treatments usually start with chemotherapy

Thalamic Tumor

  • Most are benign astrocytomas, though some are malignant
  • 80% of patients have hydrocephalus
  • May be resectable, but motor weakness and speech difficulty may occur

Symptoms

  • Hand tremors
  • Weakness of extremities
  • Headaches and nausea (related to hydrocephalus)

Treatment

  • Surgical resection for benign tumor
  • Radiation if unresectable
  • Radiation and chemotherapy if tumor is malignant