CNS tumors

Question 1

Most common malignant adult neoplasm and where is it most commonly found

Answer 1

Glioblastoma multiforme

cerebrum/ corupus callosum

Question 2

Most common benign adult neoplasm

where is if most commonly found

Answer 2

Meningioma

falx cerebri

Question 3

sites of hemangioblastoma

Answer 3

retina

cerebellum

Question 4

diagnostics of hemangioblastoma

Answer 4

sharply demarcated intraparenchymal mass

~60% cystic with non-enhancing wall

Question 5

associations of hemangioblastoma

Answer 5

VHL

Question 6

Imaging of Glioblastoma multiforme

Answer 6

garland like contrast enhancement with central necrosis

Question 7

Morphology of glioblastoma multiforme

Answer 7

hypercellular white matter with extensive astrocytic aberration

microvascular proliferation

areas of necrosis and hemorrhage with surrounding pseudopalisading arrangement of tumor cells

Question 8

associations of glioblastoma multiforme

Answer 8

overexpression of ERFR

Question 9

Where are oligodendrogliomas most commonly found

what population do you most commonly see them?

Answer 9

frontal lobe

adults in their 40-50’s

Question 10

Diagnostics of an oligodendroglioma

Answer 10

intraparenchymal tumor with calcifications

Question 11

Classic morphology of oligodendrogliomas

Answer 11

small clear cytoplasm with large round nuclei (appearing as fried eggs) and chicken wire pattern of capillary anastomoses

Question 12

Diagnostics of meningiomas

Answer 12

well defined space-occupying lesion resembling a snowball

Dura mater sign

Ginko leaf sign in the spinal cord

sunburst appearance

Question 13

Morphology of meningiomas

Answer 13

psammoma bodies

onion peel arrangements of tumor cells with whorled pattern of concentrically arranged spindle cells

Question 14

Associations of Meningiomas

Answer 14

NF2

Question 15

Location of acoustic schwannomas

Answer 15

internal auditory meatus at the cerebellar pontine angle

Question 16

Diagnostics of acoustic schwannomas

Answer 16

enhancing lesion by the internal auditory canal with possible extension into the cerebellopontine angle S100+

Question 17

Morphology of acoustic schwannomas

Answer 17

biphasic pattern of Anoni A and B bodies

Question 18

Describe Antoni A-bodies

Answer 18

spindle cells arranged in palisading patterns with interspersing nuclear-free zones (Verocay bodies)

Question 19

describe Antoni B bodies

Answer 19

paucicellular pattern with unstained myxoid stroma

Question 20

Association of bilateral schwannomas

Answer 20

NF2

Question 21

Most common malignant pediatric tumor

Answer 21

medulloblastoma

Question 22

where is medulloblastoma typically located

Answer 22

cerebellum

Question 23

Diagnostics of medulloblastoma

Answer 23

contrast-enhancing mass on imaging

Question 24

Morphology of medulloblastoma

Answer 24

sheets of small anaplastic blue cells with scant cytoplasm (primitive cells) with high mitotic figures Homer Wright Rosettes

Question 25

associations of medulloblastoma

Answer 25

Turcot syndrome

Question 26

Most common benign pediatric tumor

Answer 26

pilocytic astrocytoma

Question 27

most common location of pilocytic astrocytoma

Answer 27

**cerebellum**

Question 28

Diagnostics of pilocytic astrocytoma

Answer 28

well-demarcated cystic lesions

Question 29

Morphology of pilocytic astrocytoma

Answer 29

bipolar cells composed of *central basophilic nuclei with eosinophilic pilocytic processes* (**Rosenthal fibers)**

Question 30

Location of Epyndymoma

Answer 30

around the ventricles and floor of 4th ventricle

Question 31

are Epyndymomas malignant or benign?

Answer 31

**benign**

Question 32

Diagnostics of Epyndymomas

Answer 32

intraparenchymal tumor with **calcifications** and **cystic components** due to necrosis and/or hemorrhage

Question 33

morphology of Epyndymomas

Answer 33

**perivascular pseudorosettes** rod-shaped **blepharoblasts** near the nucleus

Question 34

Association of Epyndymoma

Answer 34

NF2

Question 35

Origin of craniopharyngiomas

Answer 35

rathkes pouch

Question 36

are craniopharyngiomas benign or malignant?

Answer 36

**benign**

Question 37

diagnostics of craniopharyngiomas

Answer 37

**suprasellar** calcified cysts with lobulated contouring

Question 38

Morphology of craniopharyngiomas

Answer 38

cyst filled with viscous brown/ yellow cholesterol cysts, **resembling motor oil** cords of palisading squamous epithelium with **wet keratin**

Question 39

clinical features of tuberous sclerosis

Answer 39

hamartomas seizures ash leaf spots shagreen patches **cysts on liver, kidney and pancreas** intellectual disability

Question 40

cause of tuberous sclerosis

Answer 40

mutation of TSC1 on chromosome 9 (codes **hamartin**) or mutation of TSC2 on chromosome 16 (codes **tuberin**)

Question 41

clinical presentation of Sturge-Weber Syndrome

Answer 41

port wine stained **facial angioma in V1 and V2** leptomeningeal angiomas early-onset glaucoma infantile epilepsy intellectual disability

Question 42

What conditions are associated with NF1

Answer 42

scoliosis pheochromocytoma Wilms tumor

Question 43

pathology of NF1

Answer 43

mutation of NF1 gene on chromosome 17 that codes for **neurofibromin**

Question 44

pathology of NF2

Answer 44

mutation of NF2 gene on chromosome 22 that codes for **merlin**

Question 45

clinical presentation of NF2

Answer 45

**bilateral acoustic schwannomas** multiple meningiomas multiple cerebral and spinal tumors

Question 46

associations of NF2

Answer 46

Meningiomas Schwannomas

Question 47

Associations of VHL

Answer 47

**hemangioblastomas** bilateral renal cell carcinomas pheochromocytoma

Question 48

clinical presentation of VHL

Answer 48

cysts in the pancreas, liver and kidneys **hemangioblastomas** in the retina, cerebellum, brain stem and spine

Question 49

pathology of VHL

Answer 49

deletion of VHL gene on **chromosome 3**, which knocks out the tumor suppressor gene

Question 50

explain the sequelae of increased HIF-1a in VHL

Answer 50

1. deletion of VHL leads to increased HIF-1a 2. increased HIF-1a leads to increased VEGF, EPO, PDGF and TGF-a 3. leads to angiogenesis 4. polycythemia and growth stimulation