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Adult-all sites Flashcards

1
Q

Which is the most prevalent adult malignant primary brain tumor? Prevalens?

A

Gliomas.
6/100 000. Arise from gliacells.

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2
Q

when was the 5th edition of WHO classification released?

A

in 2021.

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3
Q

What is the most radical change in the new classification of gliomas?

A

The are charecterized molecularly

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4
Q

How many percent of the gliomas are IDH wild type?

A

90%

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5
Q

What differs between IDH wt and mut gliomas?

A
  • unique biological features
  • treatment rsponse
  • overall survival.
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6
Q

Which is the most common type of glioma?

A

GBM glioblastoma multiforme. 53.5%

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7
Q

What is the average survival for GBM (WHO CNS 2021) under standard treatment ( maximal safe tumor resection + stupp protocol)

A

14 mo

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8
Q

What is the expected survivaltime for GBM w/o postoperative treatment?

A

4 mo

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9
Q

What is IDH involved in?

A

ezymatic alteration of the Krebs cycle, affecting critical metabolic processes.

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10
Q

What happens in IDH mutant tumors?

A

produce high levels of D-2-hydoxyglutarate -(D-H2G)

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11
Q

What changes due to high D-H2G expression make a change for the IDH mutant patients?

A

It indirectly promote gliomagenesis that render the tumor more suseptible to radiation and chemotherapy.

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12
Q

Is IDHwt or IDH mut patients more sensitive to radiotherapy?

A

IDH mutant.

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13
Q

Is IDHwt or IDH mut patients more sensitive to chemotherapy?

A

IDH mutant.

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14
Q

What is special with IDH wild type glioma?

A

They overexpress IDH1 indirectly generating resistance to cell death and promoting tumor progression. - Resistant to treatment.

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15
Q

Are gliomas with up or downregulated histone methylation more or less sensitive to treatment?

A

The ones w downregulated methylation pattern is more sensitive. (wild type)

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16
Q

What is the difference in overall survival between wt and mut Glioma (w full treatment)

A

Wild type: 14 mo
Mutated: 40 mo

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17
Q

What is required to be named GBM in the WHO-CNS5 classification?

A

IDH WILD TYPE status.

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18
Q

What more than IDH wt is needed to be called GBM?

A

One of two options:
1. histologic features suggestive of GBM

  1. 3 or more genetic parameters fullfilled ; examples: TERT promotor mut, EGFR gene amplicfication, combined gain of entire Chr 7 and loss of enire chr 10 (+7/-10)
19
Q

What is the most common non-malignant primary intracranial tumor?

A

non-malignant meningioma - 39.7% of all and 55.4% of the non-malignant.

20
Q

Genetic tumour syndromes according to WHO 2021 (CNS5)

A

kolla upp

21
Q

What are the 13 WHO CNS5 groups of tumours?

A
  1. Gliomas, glioneuronal and neuronal tumours
  2. Choroid plexus tumours
  3. Embryonal tumours
  4. Pineal tumours
  5. Cranial and paraspinal nerve tumours
  6. Meningiomas
  7. Mesenchymal, non-meningothelial tumours involving the CNS
  8. Melanocytic tumours
  9. Haematolymphoid tumours involving the CNS
  10. Germ cell tumours
  11. Tumours of the sellar region
  12. Metastases to the CNS
  13. Genetic tumour syndromes involving the CNS
22
Q

What types of tumours belong to haematolymphoid tumours?

A

*lymphomas
*histiocytic

23
Q

In case of Lymphoma, what distinction is important to make?

A

If its a primary or secondary manifestation in CNS.

24
Q

Which is the most common primary CNS lymphoma?

A

Diffuse large B-cell lymphoma of the CNS

25
Q

What tumour types has the same embryonal origin as germ cell tumours of the CNS?

A

other gonodal and extraneuraxial derivated tumours

26
Q

Name the 5 types of histiocytic tumors in WHO 2021

A

Erdheim-Chester disease
Rosai-Dorfman disease
Juvenile xanthogranuloma
Langerhans cell histiocytosis
Histiocytic sarcome

27
Q

Name the 8 types of germ cell tumors in WHO CNS 5

A

Mature teratoma
Immature teratoma
Teratoma w somatic type malignancy
germinoma
Embryonal carcinoma
Yolk sac tumor
Choriocarcinoma
Mixed germ cell tumor

28
Q

What is SEGA?

A

Subependymal giant cell astrocytoma ( a type of circumscribed astrocytic glioma)

29
Q

Differential diagnosis of tumors in the side ventricles?

A
  • central neurocytoma
  • choroid plexus papilloma
  • choroid plexus carcinoma
  • ring shaped ventricular nodules
30
Q

What are the two most common circumscribed astrocytic gliomas?

A
  1. Pilocytic astrocytoma
  2. SEGA - subependymal giant cell astrocytoma
31
Q

It should be noted that generally there is shift towards placing less importance on grade as it is often less relevant than location or available therapies. (WHO CNS 5) - name a tumor that pinpoints this aspect

A

MEdulloblastoma, WNT activated. It is grade 4, but if treated has a good prognosis far better than other medulloblastoma types.

32
Q

Anaplastic has been dropped in the new classification. What was previously known as an “anaplastic astrocytoma” is now referred to as something else. What?

A

“astrocytoma, IDH-mutant, CNS WHO grade 3”

33
Q

For the first time, molecular features have been explicitly added to the grading schema and may supersede histological features. Give an example

A

IDH-wildtype astrocytoma with low-grade histologic features can be considered grade 4 (glioblastoma) in the presence of EGFR amplification, TERT promoter mutation or the combined gain of chromosome 7 and loss of chromosome 10 [+7/-10]

34
Q

Another change in 2021 WHO is the combining of solitary fibrous tumors of the dura with another tumor type , which although appearing very different on imaging seem now to be manifestations of the same tumor. Which?

A

Hemangiopericytoma

35
Q

Which are the three types of “Choroid plexus tumors” (2nd group of tumors in WHO 2021)

A

*choroid plexus papilloma
*atypical choroid plexus papilloma
*choroid plexus carcinoma

36
Q

which tumors belong to the undergroup “Other CNS embryonal tumors”?

A
  • atypical teratoid/rhabdoid tumor
  • cribriform neuroepithelial tumor (provisional inclusion)
  • embryonal tumor with multilayered rosettes (ETMR)
  • CNS neuroblastoma, FOXR2-activated
  • CNS tumor with BCOR internal tandem duplication
  • CNS embryonal tumor
37
Q

Another word for cholesteatoma?

A

Epidermoid cyst.

38
Q

Where does epidermoid cysts arise from?

A

From ectoderm trapped within CNS.

39
Q

Name the 6 most common CNS metastases

A
  • lung cancer (small cell ca)
  • Breast ca
  • GI-tumors
  • Renal cell carcinoma
  • Malignant melanoma
  • Lymphoma
40
Q

Are secondary or primary lymphoma most common in CNS?

A

Primary lymphoma,

41
Q

What does PNET stand for?

A

Primary NeuroEctodermal Tumors

42
Q

What does DNET stand for?

A

Dysembryoplastic NeuroEpithelial Tumors

43
Q

what may paraganglioma secrete?

A

Epinephrine, Norepinephrine and Catecholamine
? stämmer detta eller är det systertumören -pheochromocytom som kan det?

44
Q

Name 5 types of paragangliomas

A

*carotid body tumor
* glomus tympanicus
* glomus jugularis
* glomus intravagale
* pheochromocytoma

Tumour (31 decks)

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