Clinical Neuropathology QUIZ #4

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Published in Clinical Neuropathology 39/6 (November/December 2020). Based on papers and editorial of Clinical Neuropathology of the same issue which you can find here: https://www.dustri.com/nc/journals-in-english/mag/clinical-neuropathology/vol/volume-39-2020/issue/novemberdecember-10.html

1. Which of the following conclusions regarding meningeal neoplasms and their inflammatory environment is valid? *

1 point

a. - PD-L1 expression is found in the vast majority of meningiomas

b - PD-L1 expression increases with tumor grade

c - Meningeal tumors are a promising target for treatment with PD-L1 inhibitors

d - Immunomodulatory mechanisms may be involved in tumor progression

e - There is no lymphocytic infiltrate in solitary fibrous tumours/hemangiopericytoma

2. Lymph vessels *

1 point

a - can be demonstrated by D2-40 (podoplanin) staining in solitary fibrous tumors/hemangiopericytomas

b - are increasingly present with increasing grade in meningiomas

c - could be demonstrated in some meningiomas

d - are associated with D2-40-positive fibroblasts

e - have been known to exist in central nervous system tumours for many years

3. Basophilic invasion *

1 point

a - is defined as the presence of corticotrophs extending into the posterior pituitary gland

b - can be demonstrated in autopsy brains with frontotemporal lobar degeneration

c - is a diagnostic feature of Alzheimer's Disease

d - is more prevalent in lower Braak and Braak Stages of Alzheimer's Disease

e - is not a feature of normal ageing of the brain

4. The posterior lobe of the pituitary gland in autopsy cases of Alzheimer's Disease *

1 point

a - does not, as a rule, include cells of the adenohypophysis

b - displays immunoreactivity for Nestin in Herring Bodies

c - Includes corticotrophs which are positive for stem cell markers

d - usually includes tau-positive tangles

e - often has neuritic plaques in Alzheimer's Disease

5- Which of the following is a feature of the amniotic rupture sequence? *

1 point

a - digital/limb amputations

b - craniofacial clefts

c - calvarial defects

d - visceral malformations

e - all of the above

6. From the case series presented by Shannon et al. on amniotic rupture sequence, one may conclude: *

1 point

a - amniotic rupture sequence can only be diagnosed in the third trimester

b - There is a strong association of craniofacial anomalies with cerebral anomalies

c - amniotic rupture sequence is often due to an abnormal karyotype

d - frequently it co-occurs with a single umbilical artery

e - The CNS anomalies seen in amniotic rupture sequence are exclusively a consequence of mechanical stress on the cranium

7. Angiocentric Gliomas *

1 point

a - often occur in young people

b - frequently present with epilepsy

c - are usually located in the cerebral hemispheres

d - have perivascular rosettes on histologic examination

e - all of the above

8. Which of the following molecular alteration is very commonly found in angiocentric glioma *

1 point

a - TERT promoter mutation

b - BRAF V600E mutation

c - FGFR alteration

d - MYC amplification

e - MYB-QK1 rearrangement

9. Oligoastrocytoma *

1 point

a - has a dual genotype in very few cases

b - is composed of two morphologically distinct neoplastic cell populations

c - corresponds histologically to WHO grade II

d - is considered as exceptional type of lesion in the WHO 2016 classification

e - all of the above

10. Which of the following statements regarding amyotrophic lateral sclerosis is correct? *

1 point

a - frequently presents with orthostatic hypotension

b - familial forms due to a mutation in the VABP gene feature FUS-positive cytoplasmic inclusions

c - the cellular mechanisms leading to neuronal loss in ALS are well known

d - features small fiber neuropathy significantly more often than controls

e - Autonomic dysfunction is usually not found in patients with ALS associated with mutations in the SOD1 gene

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