Clinical Neuropathology QUIZ #22

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Published in Clinical Neuropathology Vol 42 nr. 1, 2024. Based on papers and editorial of Clinical Neuropathology of the same issue: https://www.dustri.com/nc/journals-in-english/mag/clinical-neuropathology/vol/volume-43-2024/issue/januaryfebruary-12.html

1. Which of the following is the most common SWI/SNF-deficient tumor of the central nervous system?

1 point

a - SMARCA4-deficient AT/RT

b - AT/RT of the sellar region

c - Low-grade diffusely infiltrative tumor (LGDIT), SMARCB1-mutant

d - SMARB1-deficient AT/RT

e - Desmoplastic myxoid tumor (DMT), SMARCB1-mutant

2. Which of the following statements regarding SWI/SNF-deficent tumors of the central nervous system is NOT correct?

1 point

a - This tumor group includes high-grade and low-grade tumors

b - The morphological spectrum of this tumor group is broad

c - Some of these tumors occur in the context of a pathogenic SMARCB1 or SMARCA4 germline variant

d - These tumors occur exclusively in infants and children

e - Tumor progression from low-grade to high-grade is possible

3. What is the etiology of SMART syndrome and focal neuronal gigantism (FNG)?

1 point

a - Molecular alterations in the MAP kinase pathway

b - A paraneoplastic condition

c - A neurodevelopmental condition

d - A metabolic disorder

e - Radiation to the brain

4. Which of the follwoing histological features can be observed both in SMART syndrome as well as in FNG? *

1 point

a - Disordered neuronal Nissl substance

b - Gliosis

c - Neuronal gigantism

d - Abnormal cortical architecture

e - All of the above

5. Which of the following immunohistochemical markers is expressed in chordoid glioma? *

1 point

a - Neurofilament protein

b - Brachyury

c - TTF1

d - CD31

e - Synaptophysin

6. Which of the following mutations is a diagnostic hallmark of chordoid glioma? *

1 point

a - H3 p.K28M

b - BRAFV600E

c - PRKCA p.D463H

d - KLF p.K409Q

e - PDGFRA p.K385L

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