neurocutaneous diseases / brain tumor

Question 1

neurocutaneous disorders - type

Answer 1

1. Sturge-Weber syndrome
2. Tuberous sclerosis
3. Neurofibromatosis type 1 (von Recklinhhausen disease)
4. von Hippel-Lindau disease

Question 2

von Hippel-Lindau disease - manifestations

Answer 2

1. hemangioblastomas in retina, brain stem, cerebellum, spine
2. angiomatosis (cavernous hemangiomas in skin, mucosa, orgnas)
3. bilateral renal cell carcinoma
4. pheochromocytomas

Question 3

Neurofibromatosis type 1 - manifestation

Answer 3

1. cafe-au-lait spots
2. Lisch nodules (pigmented iris hamartomas )
3. cutaneous neurofibromas: soft, flesh-clored lesions attached to peripheral nerves)
4. optic gliomas
5. pheochromocytomas
6. meningiomas and gliomas

Question 4

Neurofibromatosis type 1 (von Recklinghausen Disease)

Answer 4

• There is no specific treatment

- Eight CN lesions may need surgical decompression to help preserve hearing

Question 5

tuberous sclerosis - manifestations

Answer 5

1. Hamartomas (in CNS and skin) 2. Angiofibromas
2. Mitral regurgitation 4. ash leaf spots 5. cardiac rhabdomyomas 5. Metal retardation 6. renal angimyolipoma 7. seizures 8. Shangreen patches
3. high incidence of subependymal astrocytomas and ungual fibromas
   ADENIMA SEBACUM (REDDENED FACIAL NODULES)

Question 6

tuberous sclerosis - increased incidence of

Answer 6

subependymal astrocytomas and ungual fibromas

Question 7

tuberous sclerosis - treatment

Answer 7

no specific treatment

control seizures g

Question 8

Struge - weber syndrome - manifestation

Answer 8

1. nevus flammeus 2. ipsilateral leptomeningeal angioma 3. intellectual disability 4. episcleral hemangioma
2. early onset glaucoma 6. epilepsy/seizures
   - -> seizures, homonymous hemianopasia, hemiparesis

Question 9

Struge - weber syndrome - image + treatment

Answer 9

image: skull x-ray shos calcifications of angiomas

to treatment / control seizures

Question 10

Adult primary brain tumors - types

Answer 10

1. Glioblastoma multiforme (grade IV astrocytoma)
2. Meningioma
3. Hemangioblastoma
4. Schwannoma
5. Oligodendroglioma
6. Pituitary adenoma

Question 11

Childhood primary brain tumors - types

Answer 11

1. Pilocytic (low grade) astrocytoma
2. Medulloblastoma
3. Ependymoma
4. Craniopharyngioma
5. pinealoma

Question 12

Glioblastoma multiforme - median survival / behavioral / frequency / found in

Answer 12

median surv: 1 year
behavioral: highly malignant primary brain tumor
frequency: common
found in cerebral hemisphere
can cross corpus callosum (butterfly glioma)

Question 13

Hemangioblastoma - most often area / can produce / associated with

Answer 13

cerebellum
EPO
VHL

Question 14

pilocytic (low grade) astrocytoma - benign or malignant?

prognosis? / area

Answer 14

Benign / good prognosis
most often found in posterior fossa (eg. cerebellum)
May be supratentorial

Question 15

ependymoma - area / prognosis

Answer 15

most commonly found in 4th ventricle

poor prognosis

Question 16

Most common supratentorial tumor in childhood / symptoms

Answer 16

craniopharyngioma:
1. bitemporal hemianopia
2. hypopituitarism
3. hyperphagia (destruction of ventromedial area - satiety area of hypothalamus)

Question 17

Medulloblastoma - benign or malignant / area / manifestations

Answer 17

highly malignant 
area cerebellum 
manifestations
1. compress 4th ventricle causing hydrocephalus 
2. send "drop metastases" to spinal cord

Question 18

Pinelioma can cause … (and mechanism)

Answer 18

1. Parinaud syndrome: compression of tectum –> ertical gaze palsy
2. obstructive hydrocephalus (compression of cerebral aqueduct)
3. precosious puberty in males (increased β-HCG production)

Question 19

…. is the most common primary malignant central nervous system (CNS) tumor arising in children.

Answer 19

Medulloblastoma