Glioma, glioneuronal and neuronal tumors

Question 1

which are the glioma, glioneuronal and neuronal tumors?

Answer 1

• diffuse glioma of the adult
• pediatric diffuse low-grade glioma
• pediatric diffuse high-grade glioma
• circumscribed astrocytic glioma
• glioneuronal and neuronal tumors
• ependymal tumors.

Question 2

About how many incidenses per 100000 people is there of high malignant astrocytoma per year in sweden?

Answer 2

5

Question 3

What makes a tumor diffuse astrocytoma vs oligodendroglioma?

Answer 3

The IDH status.

Question 4

What is IDH?

Question 5

What is needed for a IDH mutated astrocytoma to be an astrocytoma grade 4?

Answer 5

• Loss of ATRX
• homozygous CDKN2A/B deletion

Question 6

Vad heter den tumörtyp som tidigare kallades sekundärt gliom?

Answer 6

Astrocytoma grade 4

Question 7

What is the most common MALIGNANT braintumor in the adults?

Answer 7

Glioblastoma - 35% of all primary (malignant?) brain tumors.

Question 8

What is the normal age span of debute w high malignant glioma?

Answer 8

55-75 yo

Question 9

Which, unkommon, genetic syndromes are associated with a higher risk of glioma?

Answer 9

Li Fraumeni
Turcots
NF
Von Hippel Lindaus

Question 10

What are the differential diagnostics to glioma?

Answer 10

• brain mets
• primary CNS lymfoma
• Meningioma
• Brain abscess
• Neurosarcoidosis
• Dementia

Question 11

What is the advantages of methylated MGMT promotor?

Answer 11

• better prognosis
• Better effect of alkylating cyto - Temozolomid.

Question 12

What does pseudoprogression mean?

Answer 12

In 20-30% of patients treated with radiotherapy w/wo Themozolomide treatment, there will be a contrastenhancement 1-3 months after finished treatment caused by reactive changes due o the treatment.

Question 13

How can a pseudoprogression be destinguished?

Answer 13

Perfusion MRT with high CBV in the same area as the Gd attenuation speaks for new tumor. The risk of pseudoattenuation is lower 3-4 months after treatment.

Question 14

There is another way to differentiate between pseudoprogress and truth tumor. Which?

Answer 14

Aminoacid-PET.

Question 15

Which are the adult-type diffuse gliomas?

Answer 15

• Astrocytoma IDH mutant
• Oligodendroglioma, IDH mutant and 1p/19q-codeleted
• Glioblastoma, IDH-wildtype

Question 16

After being assessed as “Astrocytoma IDH mut”, grading is going to be ashived. How is that done?

Answer 16

Its unchanged compared to WHO CNS 2016.
* Necrosis and/or microvascular proliferation = grade 4
* Ki-67/MIB-1 proliferative index significantlt increase w grade but no cutoff is established for grade 2 and 3.
* If a CDKN2A/B homozygous deletion is found = grade 4.

Question 17

For adult diffuse gliomas, what are prognostic factors?

Answer 17

• young age +
• higher KPS +
• MGMT promotor methylation +
• large postoperative residual tumor -

Question 18

Why are 1p/19q codeletion and IDH mutation not a positive prognostic factor since 2016?

Answer 18

They are now disease defining features and therefore no longer prognostic within a given subtype.

Question 19

Does it matter to overall survival if postsurgical radiotherapy of gliomas is started less than 3 weeks from surgery or between 3-5 weeks?

Answer 19

No.