Neuro-oncology

Question 1

What is the most common primary brain tumor?

Answer 1

Meningioma

Question 2

Differentiate high and low grade glioma based on MRI

Answer 2

Low: Non enhancing on T1 need FLAIR or T2 to see full picture
High: Enhancing intra-axial mass lesions

Question 3

What is the first choice steroid to relieve headache and lateralizing signs?

Answer 3

Dexamethasone

Question 4

Where are true rosettes and pseudorosettes seen?

Answer 4

T: Flexner-Wintersteiner– Retinoblastoma
F: Homer Wright– Neuroblastoma, Medulloblastoma
Perivascular pseudorosettes also seen in ependymomas

Question 5

Where are rosenthal fibers seen?

Answer 5

Pilocytic astrocytoma but also seen as “reactive Rosenthal fibers” in craniopharyngioma, hemangioblastoma and pineal cysts

Question 6

What are the 2 pathologic features either of which is required to make a diffuse astrocytoma a grade 4– glioblastoma?

Answer 6

Microvascular proliferation

Necrosis

Question 7

What low grade glioma needs more aggressive treatment because of the worse prognosis associated with it?

Answer 7

Gemistocytic low grade astrocytoma

Question 8

What are the pathologic feature is required to make a diffuse astrocytoma a grade 3– anaplastic astrocytoma?

Answer 8

Mitosis

Question 9

What feature makes a GBM tumor responsive to Temozolomide, a DNA alkylating agent?

Answer 9

DNA repair enzyme (MGMT– methylguanine DNA methyltransferase) methylation (inactivation)

Question 10

What is the key pathologic description of oligodendroglial tumors?

Answer 10

Uniform “fried egg” appearance, due to perinuclear cytoplasmic clearing

Question 11

What is the most common primary spinal cord tumor?

Answer 11

Ependymoma

Question 12

Gangliocytoma and dysplastic gangliocytoma, hamartomatous cerebellar lesion with mature ganglion cells are associated with a mutation in which gene?

Answer 12

PTEN, Cowden syndrome

These tumors are called Lhermitte-Duclos dz

Question 13

Central neurocytoma is located in?

Answer 13

Intraventricular –> Hydrocephalus!

Question 14

What is the only malignant neuronal tumor?

Answer 14

Neuroblastoma

Question 15

What is a marker of neuronal differentiation that may be utilized to identify neuronal tumors?

Answer 15

Synaptophysin

Question 16

What 3 intracranial neoplasms have a characteristic cyst with enhancing mural nodule?

Answer 16

Pilocytic astrocytoma
Ganglioglioma
Hemangioblastoma

Question 17

In stark contrast to neuronal/ neuroglial tumors embryonal tumors are all _______?

Answer 17

Malignant and highly aggressive

Question 18

What is the typical pathologic description of infratentorial PNET/ medulloblastoma?

Answer 18

Small blue cells with Homer Wright rosettes

Question 19

What are the 3 most common tumors in the pineal region? Which one of them is VERY sensitive to radiation?

Answer 19

Germinoma > Pineal parenchymal tumors > Astrocytoma

Germinoma sensitive to radio

Question 20

Which is malignant? Pineocytoma or Pineoblastoma?

Answer 20

Pineoblastoma! Needs aggressive multiagen chemotherapy while pineocytoma only needs resection!

Question 21

What stain can be used to identify germinomas?

Answer 21

Alkaline phosphatase

Question 22

What is the most common locations of meningiomas?

Answer 22

Cerebral convexities

Question 23

What are the 4 aggressive histologic subtypes of meningioma?

Answer 23

2CPR

Clear cell, Choroid, Papillary and Rhabdoid

Question 24

What are the ddx for dural based lesions?

Answer 24

Meningioma
Hemangiopericytoma
Dural mets

Question 25

Why is it important to differentiate meningioma from a hemangiopericytoma?

Answer 25

Hemangiopericytoma needs RT while meningioma only needs resection in most cases

Question 26

What is the characteristic pathologic description of hemangiopericytoma?

Answer 26

Staghorn pattern of typical branching vascular pattern

Question 27

What mutation leads to bilateral vestibular schwannomas?

Answer 27

NF2 gene on chromosome 22

Question 28

What pathologic features may be seen in Schwannomas?

Answer 28

Antoni A regions with dense cellular componene and Verocay bodies, organized linear palisades of nuclei

Question 29

Differentiate NF1 and NF2 based on the tumors associated with them. Which one has Lisch nodules in the iris?

Answer 29

1: NO-- Neurofibroma and Optic glioma 2: SMEG-- Schwannoma, Meningioma, Ependymoma, Glioma RARELY Lisch: 1

Question 30

What is the pathologic picture of primary malignant lymphomas?

Answer 30

Perivascular cuffing by malignant lymphocytes which are CD 20 positive B cells

Question 31

What is the treatment for primary CNS lymphoma?

Answer 31

High dose methotrexate based chemo regimen

Question 32

What endocrine symptoms are seen in large nonsecreting sellar tumors?

Answer 32

Hyperprolactinemia and hypopituitarism

Question 33

What is the most common finding in pituitary macroadenoma?

Answer 33

Bitemporal hemianopsia

Question 34

What diagnostic exams are used to identify endocrinologic derangements in pituitary microadenomas?

Answer 34

Serum prolactin >200ng/ml Serum IGF 1 24 hour urine cortisol excretino and ACTH concentration

Question 35

What is the pathologic description of craniopharyngioma?

Answer 35

Crank-case oil fluid in a cyst arising from the Rathke's Cleft

Question 36

What is treatment for Craniopharyngioma?

Answer 36

Sx then RT

Question 37

What are the ddx for intraventricular tumors?

Answer 37

``` Meningioma Neurocytoma subependymal giant cell Astrocytoma Subependymoma Ependymoma CHoroid plexus papilloma MAN ChES ```

Question 38

What does a Choroid plexus papilloma look like on imaging?

Answer 38

Calcified enhancing intraventricular mass

Question 39

What is the most common location of an CNS hemangioblastoma?

Answer 39

Cerebellum or SC

Question 40

Name the cancer syndromes associated with the ff CNS tumors: Cortical hamartomas Subependymal giant cell Astrocytoma SEGA Hemangioblastoma Astrocytoma Gangliocytoma of the cerebellum (Lhermitte-Duclos) Medulloblastoma

Answer 40

Cortical hamartomas-- TUBEROUS SCLEROSIS Subependymal giant cell Astrocytoma SEGA -- TUBEROUS SCLEROSIS Hemangioblastoma-- VHL Astrocytoma-- LI FRAUMENI Gangliocytoma of the cerebellum (Lhermitte-Duclos)-- COWDEN Medulloblastoma -- TURCOT

Question 41

What cancer has _____ as paraneoplastic syndrome? Limbic encephalitis Stiff man syndrome Lambert eaton syndrome

Answer 41

ALL Small Cell Lung Cancer