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Flashcards in Brain tumors Deck (47)
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1

What is the differential diagnosis of a spine tumor (3)?

1) Low grade astrocytomas
2) Ependymomas
3) AA/GBM (much less commonly)

2

What is the differential diagnosis of a posterior fossa tumor (5)?

1) Pilocytic astrocytoma
2) Medulloblastoma
3) Ependymoma
4) ATRT
5) DIPG

3

How would you differentiate on imaging a brainstem diffuse midline glioma vs pilocytic astrocytoma?

DIPG (diffuse midline glioma H3K27M mutant): diffuse, commonly but not always in the pons, encasing basilar artery
PA: exophytic, bright post-gad, can occur anywhere in the brainsteam

4

Which brain tumors don't need to be biopsied for Dx?

1) NG-GCT (if positive tumor markers)
2) Diffuse midline glioma H3K27M mutant, unless atypical features

5

Which brain tumors are usually not considered for radiation therapy?

1) Choroid plexus carcinoma
2) LGG, unless failure of other options

6

What is the common radiological appearance of pilocytic astrocytoma?

Most often cerebellar location (but can happen anywhere), contrast-enhancing, bright on T2, classically nodule with cyst

7

What targeted therapy could be considered if...
a) BRAF fusion
b) BRAF V600E mutation

a) MEK inhibitor e.g. trametinib
b) BRAF inhibitor

8

Staging: LGG and HGG

MRI of brain +/- spine
No metastatic potential outside the CNS

9

Prognosis of HGG

Anaplastic astrocytoma: 30% (GTR and adjuvant chemo)
GBM: EFS 18%

10

What is the OS for
a) Germinoma

b) NGGCT

a) Germinoma:
OS>90%

b) NGGCT
OS 70%

11

What is growing teratoma syndrome?

Syndrome associated with NGGCT(pathology invariably mature teratoma)

Rapid radiological enlargement with progressive decrease of tumor markers

Treatment: surgical resection, ideally gross total extirpation followed by RT

12

Germinoma: treatment

Localized:
Chemotherapy (carboplatin-etoposide, or ifosphamide-etoposide-carboplatin) followed by radiation (whole ventricular RT 24Gy with tumor boost 16Gy)
COG testing lower RT dose
In new trial

Metastatic:
Chemotherapy (carboplatin-etoposide, or ifosphamide-etoposide-carboplatin) followed by radiation (craniospinal RT with tumor boost)

13

Treatment: NGGCT

- Chemotherapy (platinum based, for example ifos-carbo-etoposide)
followed by
- RT: volume and dose debated; COG does CSI at 36Gy with tumor boost

- Surgical excision for mature teratoma and benign immature teratoma

14

Epidemiology of GCT (CNS)
a) Histologic subtypes

b) Sex predominance

A) 60-70% Germinomas
30-40% NGGCT

b) Male prédominance in general
Pineal region: 15M:1F
Suprasellar region: slight female predominance

15

RF for brain tumors

- Ionizing radiation (meningioma, HGG, sarcoma)
- Immunosuppression (CNS lymphoma)
- Specific genetic conditions (differs for each brain tumors)
No proven environmental factor

16

Describe histologic features PA

- Rosenthal fibers
- Eosinophilic granular bodies
- GFAP (+)ve
- Low mitosis rate (MIB1 < 4%)

17

LGG: prognosis

OS: very good (>90%)
PFS: depends on the location; about 50% in unresectable lesions

18

Classical presentation of optic pathway glioma

- Visual loss
- Proptosis
- Optic nerve atrophy
Clinical course very variable, usually more indolent in NF1-OPG

19

OPG: treatment considerations

- First line of treatment = chemotherapy
re; risk associated with surgery
VCR/carbo and TPCV have shown similar efficacy
- TPCV shouldn't be used in NF1 patients re: risk of malignancies with alkylators

20

HGG: prognosis as per grade and degree of resection

Anaplastic astrocytoma:
With GTR: 44%
W/O GTR: 22%
GBM:
With GTR: 26%
W/O GTR: 4%

21

ATRT: typical histology

Eosinophilic nucleus, with prominent eosinophilic nucleoli, abundant cytoplasm, and eosinophilic globular cytoplasmic inclusion

Histology might be misleading (e.g. areas of small round blue cells), so diagnostic mostly based on loss of SMARCB1 (FISH or immunostaining)

22

Prognostic factors: HGG

- WHO grade
- Gross total resection
- Biologic markers
(+)ve: PTEN, MGMT methylation, (-)ve: p53, endoglin, PARP positivity
MIB1 (mitosis rate)

23

DIPG: what treatment options are available in case of reprogression?

Only repeat radiation therapy have shown effect

24

What mutation is often encountered in craniopharyngioma?

Papillary subtype: BRAF v600E
Adamantinomatous: b-catenin

25

Craniopharyngioma: standard treatment for newly diagnosed CPG

- Resection, total if possible
- Radiation therapy if subtotal resection can be considered
- Intracystic sclerosing agents (interferon, bleomycin) can be used as temporizing agents for the cystic part

26

ATRT: typical histology

Eosinophilic nucelus, with prominent eosinophilic nucleoli, abundant cytoplasm, and eosinophilic globular cytoplasmic inclusion

Histology might be misleading (e.g. areas of small round blue cells), so diagnostic mostly based on loss of SMARCB1 (FISH or immunostaining)

27

Epidemiology of ATRT

1-2% of childhood brain tumors
Male predominance, mostly in infants < 3 y.o.

28

WHO classification of choroid plexus tumors

WHO grade I = CP papilloma; closely resemble normal CP with low proliferation rate
WHO grade II = atypical papilloma; CPP with increased mitotic activity
WHO grade III = CP carcinoma; higher cell density, freq mitosis, high N/C ratio, necrosis, invasive appearance

29

Ependymomas: unfavorable prognostic factors (7), as per cancer.gov

- Younger age
- Higher proliferation rate (Ki67, MIB1)
- Location: cranial < spinal (lower) < spinal (higher)
- Subtotal resection
- Anaplastic histology
- Lower doses of radiation therapy (should be at least 54Gy)
- Genomic expression profile (e.g. subgroup A in posterior fossa ependymoma)

30

CP carcinoma: what is the standard treatment

Maximal surgical resection
Usually with chemotherapy (re: mostly infants < 3 months); no SOC of care but multiagent chemo such as carboplatin-etoposide has shown good activity