Lecture 4: Tumores of the CNS Part II

Question 1

What is significance of CNS neoplasms?

Answer 1

Second most common cancer in children under 15 y.o (second to leukemia)

Question 2

What is the age distribution of brain tumors?

Answer 2

Bimodal distribution
Peak incidence in children younger than 5
Example: medulloblastoma, pilocytic astrocytoma
Second larger peak in adults from 45-70
(glioblastoma, metastatic carcinoma)
Meningiomas = females
Gliomas/embryomas = males

Question 3

What are brain tumors strongly associated with?

Answer 3

Ionizing radiation (x-irradiation) from X-rays and CT scans?
Congenital/acquired immunodeficiency
-iatrogenic immunosuppression
-hematologic malignancies under treatment
-AIDS
-autoimmune diseases

Not associated with

- electrical/magnetic fields
- cell phones
- smoking
- trauma

Question 4

What are the manifestations of tuberous sclerosis? Genetics?

Answer 4

1. cutaneous angiofibroma (looks like potato?)
2. subungual fibromas
3. cardiac rhabdomyomas
4. intestinal polyps
5. renal agniomyolipomas
   Chromsome 9 and 16…TSC1/TSC2 gene

Question 5

What is the gene locus for NF1?

Answer 5

17q11

Question 6

What is the gene locus for NF2

Answer 6

22q12

Question 7

What is the significance of Li-Fraumeni syndrome?

Answer 7

It is a type of glioma (secondary GBM?)
-it “doubles up” after you get lung cancer?
Associated with other cancers
Anything with “syndrome” is associated with other cancers
17p13 at gene TP53
LEADS TO CARCINOMA

Question 8

What are the Different types of symptoms/signs depending on tumor location?

Answer 8

i. epilepsy (focal/generalized)
ii. focal neurological deficit
iii. mental changes
- tumor in hippocampus = schizophrenia
iv. obstructive hydrocephalus (headache)

Question 9

What are the symptoms of increased intracranial pressure?

Answer 9

i. headache (postural, nocturnal, early morning)
ii. vomiting (particularly in children…of course!)
iii. papilledema
iv. clouding of consciousness and coma

Question 10

How do you differentiate between psychogenic headache (due to stress) or neoplastic headache?

Answer 10

Psychogenic headache = unremitting over long peeriods of time…described with a shitload of metaphor
Neoplastic headache are aggravated by nausea and vomiting

Question 11

What brain tumor is associated with immunodeficiency?

Answer 11

Primary CNS lymphomas

From AIDS or transplant

Question 12

How do you get herniation with brain tumors?

Answer 12

Herniation seen as sequelae to raised intracranial pressure

Question 13

What are the different sites of herniation?

Answer 13

i. Subfalcine herniation
ii. Transtentorial herniation of the parahippocampal gyrus
- UNCALherniation
iii. Bilateral (central) transtentorial or diencephalic herniation
iv. Tonsillar herniation
v. Superior cerebellar herniation

Question 14

What is a duret hemorrhage?

Answer 14

Small areas of bleeding in the ventral and paramedian parts of the upper brainstem

Question 15

What are potential consequences of herniation?

Answer 15

1. blood vessels compression

2. nerves compression

Question 16

Question: A 37 year old female was diagnosed with bilateral acoustic-vestibular scwannoma (CN 8). Which familial syndrome does she have?

Answer 16

Neurofibromatosis 2 (NF2)

Question 17

A 43 year old man has a superficial oligodendroglioma of the left frontal lobe with infiltration of the cortex. The most likely presenting symptoms are:

Answer 17

Epileptiform fits