CNS Tumors

Question 1

What proportion of childhood cancer is made up of CNS tumors?

Answer 1

15-20%

Question 2

What is the most common solid neoplasm of childhood?

Answer 2

CNS tumors

Question 3

What is the overall prognosis for children diagnosed with brain tumors and treated with current treatment regimens?

Answer 3

~65% of children survive into adulthood

Question 4

Neurofibromatosis Type 1 is associated with which type of CNS tumors?

Answer 4

Primarily optic gliomas, but can also include meningiomas, ependymomas, neurosarcomas of the cranial nerves, and spinal cord astrocytomas.

Question 5

Which tumor is characteristically associated with Neurofibromatosis Type 2?

Answer 5

NF2 is characteristically associated with bilateral vestibular schwannomas.

Question 6

What are some CNS tumors that can be less commonly associated with NF2?

Answer 6

Retinal gliomas, meningiomas, gliomas, and cranial and perpheral nerve schwannomas.

Question 7

What type of tumor is most commonly seen with tuberous sclerosis?

Answer 7

Subependymal giant cell tumor, which arises in the midline.

Question 8

With what condition is a midline subependymal giant cell tumor associated?

Answer 8

Tuberous sclerosis

Question 9

What is the expected behavior of a subependymal giant cell tumor?

Answer 9

In and of itself it’s typically benign, but it can grow quite large and produce pathology due to impingement on other structures.

Question 10

What is Li-Fraumeni syndrome, and what CNS tumors are patients at an increased risk for developing?

Answer 10

It is a familial cancer syndrome that leads to an increased risk of gliomas, ependymomas, and choroid plexus carcinomas.

Question 11

What is Turcot syndrome?

Answer 11

Patients with this syndrome have an increased risk of developing glioblastoma multiforme and medulloblastoma.

Question 12

Which syndrome is characterized by an increased risk for developing glioblastoma multiforme and medulloblastoma?

Answer 12

Turcot syndrome

Question 13

What type of tumor is seen in von Hippel-Lindau disease?

Answer 13

von Hippel-Lindau disease increases the risk of developing hemangioblastomas in the cerebellum, medulla, and spinal cord.

Question 14

Fill in the blanks on Figure 25-1 with common brain tumor locations. ***

Question 15

Which cranial nerve finding is commonly seen in children with brain tumors?

Answer 15

Diplopia due to CN 6 palsy.

Question 16

What clinical finding is important to look for in an infant you suspect of having a brain tumor?

Answer 16

The infant’s cranial sutures are not fused, so one should check head circumference and look for a bulging fontanelle in an infant suspected of having a brain tumor.

Question 17

What is the sun-setting sign?

Answer 17

Impairment of upward gaze in conjunction with downward deviation of the eyes. It can be an early sign of increased ICP.

Question 18

What are some common presenting signs in infants with brain tumors?

Answer 18

Irritability, anorexia, vomiting, developmental delay, motor abnormalities, bulging fontanelle, increased head circumference, and “sun setting” sign.

Question 19

What are some common presenting problems in patients with infratentorial lesions?

Answer 19

Patients often present with problems with coordination and cranial nerve dysfunction.

Question 20

What are some common presenting problems in patients with supratentorial lesions?

Answer 20

Patients often present with headaches, weakness, and seizures.

Question 21

Differentiate the presentations between infratentorial and supratentorial lesions.

Answer 21

Patients with infratentorial lesions often present with problems with coordination and cranial nerve dysfunction. Seizures are uncommon. Patients with supratentorial lesions often present with headaches, weakness, and seizures.

Question 22

Describe Parinaud syndrome.

Answer 22

Parinaud syndrome is a triad of impaired upward gaze, dilated pupils with better reactivity to accommodation than to light, and retraction or conversion nystagmus with lid retraction. It is caused by compression or infiltration of the midbrain tectum, particularly with pineal tumors.

Question 23

What symptoms can occur if a CNS tumor has spread to the leptomeninges?

Answer 23

Intermittent mental status changes; neck, back, or radicular pain; weakness; and bowel and/or bladder dysfunction.

Question 24

What is the mainstay of therapy for most CNS tumors?

Answer 24

Surgical resection is the mainstay of therapy for most tumors of the CNS.

Question 25

Which age group normally is not treated with CNS radiation therapy due to increased risk of toxicity?

Answer 25

Due to the significant acute and long-term toxicity, radiation therapy is usually avoided in children <3 years of age.

Question 26

What are the most common type of malignant CNS tumor in childhood?

Answer 26

Primitive neuroectodermal tumors, with the majority being medulloblastomas.

Question 27

What are the most common presentations of medulloblastoma?

Answer 27

Children with medulloblastoma typically present with morning headache, vomiting, and lethargy. Ataxia is common and involves the trunk or limbs. Head tilt can occur due to 4th CN dysfunction or impending cerebellar herniation. Most patients are symptomatic for <3 months before the diagnosis is made.

Question 28

What worsens prognosis in patients with medulloblastomas?

Answer 28

Younger age, disseminated disease, brainstem infiltration, larger tumor sizes, and certain histologic and cytologic tumor features.

Question 29

How is medulloblastoma usually treated?

Answer 29

Tumor resection is first-line, with subsequent radiation. Chemotherapy is also used frequently in those with high-risk disease.

Question 30

If ependymoma tumors involve the 4th ventricle, what complication can occur?

Answer 30

If the tumor is in the 4th ventricle, CSF flow is blocked, with accompanying symptoms of nausea, vomiting, morning headache, and diplopia.

Question 31

How is ependymoma usually treated?

Answer 31

Surgery is the mainstay of therapy, with ease of resection correlating with chance of cure. Postoperative radiation seems to increase overall survival, but chemotherapy is not beneficial.

Question 32

What is the most common type of primary CNS tumor in children?

Answer 32

Gliomas make up 50-60% of brain tumors in children and are the most common primary childhood CNS tumors.

Question 33

What are the most common posterior fossa tumors of childhood?

Answer 33

Cerebellar astrocytomas

Question 34

Describe the clinical findings in cerebellar astrocytoma tumors.

Answer 34

Symptoms include clumsiness, unsteadiness of the arms and legs, headaches, and vomiting.

Question 35

How are cerebellar astrocytomas usually treated?

Answer 35

Surgery, radiation, and chemotherapy may be used. In children with complete resection of the tumor, radiation and chemotherapy may not be required.

Question 36

When is peak incidence for brainstem glioma and what is the prognosis?

Answer 36

Peak incidence is between 5 and 8 years of age. Prognosis is poor, especially for children with diffuse, infiltrating lesions.

Question 37

Which type of astrocytoma has the most aggressive clinical behavior?

Answer 37

Glioblastoma multiforme

Question 38

Describe the prevalence of astrocytoma and high-grade astrocytoma among children with brain tumors.

Answer 38

Astrocytomas account for 40% of all childhood brain tumors, with 25% of these being aggressive or high-grade.

Question 39

What is the usual treatment for high-grade astrocytoma?

Answer 39

Surgical resection is first-line, but high-grade astrocytomas generally infiltrate the brain and can’t be completely excised. Radiation may improve survival. High-dose chemo and autologous peripheral blood stem cell rescue have shown promise but outcomes remain poor.

Question 40

How to pineal tumors present?

Answer 40

Pineal tumos present with Parinaud syndrome (impaired upward gaze, dilated pupils that react better to accommodation than to light, and retraction/conversion nystagmus with lid retraction).

Question 41

How do suprasellar germinomas present?

Answer 41

They produce pituitary and hypothalamic dysfunction, such as growth hormone failure and diabetes insipidus.

Question 42

What is a common imaging finding in CNS teratomas?

Answer 42

Calcium deposits

Question 43

What tumor markers would one expect to be elevated in a patient with a mixed germ cell tumor?

Answer 43

Alpha-fetoprotein (AFP) and beta human chorionic gonadotropin (β-hCG)

Question 44

What structures are commonly affected by craniopharyngiomas?

Answer 44

Craniopharyngiomas are locally invasive and can affect the optic chiasm, carotid arteries, CN 3, and pituitary stalk.

Question 45

Name some of the complications from craniopharyngiomas.

Answer 45

> 50% of children have visual changes from optic involvement. Changes in personality and sleep patterns are common. Because of pituitary involvement, endocrinologic signs (such as growth failure, short stature, and polydipsia) are also common.

Question 46

What imaging finding is present in most cases of craniopharyngioma?

Answer 46

Calcifications in the suprasellar region are present in most cases.

Question 47

How do most choroid plexus tumors present?

Answer 47

80% appear before the 2nd birthday and most present with signs and symptoms of hydrocephalus because of excess CSF production or blockage of CSF flow.

Question 48

How are choroid plexus tumors treated?

Answer 48

Surgical resection is curative.

Question 49

Meningiomas are more common in which patients?

Answer 49

Meningiomas are rare except in patients with Neurofibromatosis Type 2 and long term survivors of other brain tumors who have received radiation therapy.

Question 50

What is the recommended treatment for meningiomas?

Answer 50

Meningiomas are almost always benign and can be cured with surgical resection.