Neuro-oncology

Question 1

Cancer associations with opsoclonus myoclonus syndrome in children?

Adults?

What antibody is sometimes associated with this in adults only?

Treatment in kids?

Answer 1

Children: neuroblastoma

Adults: breast, ovarian, SCLC

Anti-RI, especially in breast cancer

ACTH in kids (alternatives include steroids which is usually used in adults, and other immunosuppression like IVIG)
(Also treat malignancy)

Question 2

Tumor associated with perinecrotic pseudopalisaging?

Answer 2

Glioblastoma

(Also seen: nuclear atypia, mitoses, endothelial hyperplasia, necrosis)

Question 3

Tumor associated with “fried egg” appearance

Answer 3

Oligodendrogliomas

(“Fried egg” is cells with uniformally rounded nuclei with clear pericellular haloes)

Question 4

Tumor associated with perivascular pseudorosettes

Answer 4

Ependymomas

Question 5

Tumor assoiated with Homer-Wright rosettes

Answer 5

Medulloblastoma

Question 6

Paraneoplastic cerebellar disease antibodies, and tumors to which they are associated with (3)

Answer 6

Anti-YO: breast and ovarian (also others including Hodgkin’s and SCLC)

Anti-HU: SCLC (and others)

Anti-Tr: lymphomas

Question 7

Antibody treatment for recurrent glioblastomas

Answer 7

Bevacizumab (antibody against VEGF, anti-angiogenesis)

Question 8

Paraneoplastic syndrome associated with paraneoplastic optic neuropathy

Answer 8

Anti-CRMP5, a/w lng cancer

(Also can lead to encephalitis, cerebellar degeneration, chorea and others)

Question 9

Antibody with paraneoplastic retinal degeneration

Answer 9

Anti-recoverin aka anti-CAR

(A/w SCLC, thymoma, RCC, melanoma)

Question 10

Two antibodies associated with paraneoplastic chorea?

Answer 10

HU and CRMP5

Question 11

Mutation associated with half of anaplastic astrocytomas

Answer 11

P53 mutation

(also seen is loss of p16 and other retinoblastoma pathway genes)

Question 12

Tumor associated with 1p19q deletion

Answer 12

Oligodendrogliomas

Question 13

Most common glioma in children

Answer 13

Pilocytic astrocytomas

(Grade I tumor typically well circumscribed, often in the cerebellum where it is typically cystic with an enhancing mural nodule, but they are solid if in hypoathalamus or optic nerves)

Question 14

Grade I gliomas that can be a well-circumscribed cystic tumor with an enhancing mural nodule

Answer 14

Pilocytic astrocytoma

Question 15

Well-demarcated tumor usually in superficial cortex, most commonly temporal, that is a cyst with an enhancing mural nodule.

Answer 15

Pleomorphic xanthroastrocytoma

Question 16

Tumor associated with pleomorphic astrocytes arranges in fascicles with intercellular reticulin deposition, mesenchymal-like cells, and multinucleated giant cells

Answer 16

Pleomorphic xanthroastrocytoma

Question 17

Benign hamartomatous intraventricular tumor associated with tuberuous sclerosis

Answer 17

SEGA - subependymal giant cell astrocytoma

Question 18

“Candle gutterings” are histologically seen in…?

Answer 18

SEGA - subependymal giant cell astrocytoma

Question 19

Glial neoplasm associated with biphasic pattern of compact regions along with microcystic components, with hair-like astrocytic processes

Answer 19

Pilocytic astrocytoma

(The hair-like astrocytic processes are the pilocytic namesake)

Question 20

Genetic tumor syndrome associated with spinal ependymomas

Answer 20

NF2

Question 21

Tumor associated with synaptophysin staining

Answer 21

Neurocytoma (synaptophysin is a marker of neuronal differentiation and seen with neuronal tumors but not glial tumors, which would potentially be GFAP positive)

Question 22

CNS tumor associated with psammoma bodies

Answer 22

Meningioma (also prolactinomas)

(Psammoma body is a round collection of calcium that is also seen in other tumors outside the CNS, like papillary thyroid ca and papillary RCC,.

Question 23

Tumor with prominent capillary vasculature and stromal cells with a vacuolated cytoplasm

Answer 23

Hemangioblastoma

Question 24

What tumor syndrome is associated with hemangioblastoma?

Answer 24

VHL (but only about 25% of cases)

Question 25

Tumor with hypercellular, sheet-like proliferation of small blue cells wit high nuclear-to-cytoplasm ratio

Answer 25

Medulloblastoma

Question 26

Pineal tumor with homogenous, small uniform cells that resemble pineocytes

Answer 26

Pineocytoma

(As opposed to pineoblastoma, which show proliferation of small rounds cells, which are undifferentiated and resemble primitive neuroectodermal tumors rather than pineocytes)

Question 27

Pineal tumor with proliferation of small rounds cells, which are undifferentiated and resemble primitive neuroectodermal tumors rather than pineocytes

Answer 27

Pineoblastoma

(As opposed to pineocytoma, which is a pineal tumor with homogenous, small uniform cells that resemble pineocytes)

Question 28

T1 hyperintense cyst near the foramen of monro.

How can it present?

Answer 28

Colloid cyst

Can lead to forman of monro obstruction and therefore HA, AMS, drop attacks, acute hydro, and sudden death.

Question 29

Cyst with slight FLAIR hyperintensity and diffusion restrction

Answer 29

Epidermoid cyst (as opposed to arachnoid cysts, which contain CSF and so isointense to CSF on T2 and black on FLAIR)

Question 30

Pediatric tumor with prominent clear spaces with interspersed cells including glial cells?

Answer 30

DNET - dysembryoplastic neuroepithelial tumor

Question 31

Tumor associated with EBV in immunocompromised patients

Answer 31

Primary CNS lymphoma (typically a B cell lymphoma)

Question 32

Sellar tumor with multicystic components, with cord of epiethilial-appearing cells

Answer 32

Craniopharyngioma (from Rathke’s pouch remnants)

Question 33

Tumor with proliferation of epithelial cells in a papillary pattern lining fibrovascular cores

Answer 33

Choroid plexus papilloma (childhood gradeI tumor)

Question 34

Invasic osseodestructive dumor

Answer 34

Chordoma (most commonly clivus here they can lead to HA, neck pain, and cranial neuropathies, can also be sacrococcygeal and lead to sphincter dysfunction and pain)

Question 35

Encephalitis associated with testicular tumors in young males?

Answer 35

Anti-MA (also seen w SCLC in older adults)

(Can lead to limbic, brainstem, or diencephalon encephalitis)