CNS

Question 1

Li-Fraumeni Gene

Answer 1

TP53

Question 2

Gorlin’s (Nevoid Basal Cell Carcinoma Syndrome) Gene

Answer 2

PTCH

Question 3

Gorlin’s Syndrome CNS Tumor

Answer 3

Medulloblastoma

Question 4

TS CNS Tumor

Answer 4

SEGA (Subependymal giant cell astrocytoma)

Question 5

TS gene

Answer 5

TSC1 and TSC2

Question 6

Rhabdoid Tumor Predisposition Syndrome Gene

Answer 6

SMARCB1 and SMARCB4 (INI-1)

Question 7

Path with Rosenthal fibers

Answer 7

Pilocytic astrocytoma

Question 8

Most common mutation for a LGG

Answer 8

BRAF KIAA1549 fusion

Question 9

DIPG mutation

Answer 9

H3 K27M mutation

Question 10

DIPG classic triad

Answer 10

CN symptoms, long tract sx (hyperreflexia) and ataxia

Question 11

4 medulloblastoma subtypes

Answer 11

WNT, SHH, group 3 and group 4

Question 12

Need spinal MRI and CSF

Answer 12

Medulloblastoma, ependymoma, germ cell tumors, AT/RT

Question 13

Medulloblastoma HR features

Answer 13

Residual tumor > 1.5cm^3, metastatic diseae, anaplastic/large cell histology

Question 14

Ependymoma path

Answer 14

Perivascular pseudorosettes

Question 15

Ependymoma molecular biology

Answer 15

EPN-YAP 1 = good, EPN-RELA = poor

Question 16

Latency period for secondary CNS tumors after radiation

Answer 16

7-10 years