25 Cancers of the Central Nervous System

Tanner M. Johanns, Jian Li Campian and George Ansstas

QUESTIONS

Each of the numbered items below is followed by lettered answers. Select the ONE lettered answer that is BEST in each case unless instructed otherwise.

Question 25.1 Linkage studies have identified genes associated with neurofibromatosis type 2 (NF2), Turcot syndrome, and Li–Fraumeni syndrome in which of the following chromosomes?

A. 10q, 22q, and 17q

B. 22q, 5q, and 17p

C. 5q, 10q, and 17p

D. 5q, 10q, and 17q

Question 25.2 Of the following, the most common primary brain tumor in adults in the United States is:

A. Glioblastoma.

B. Meningioma.

C. Astrocytoma.

D. Oligodendroglioma.

Question 25.3 The gene that is frequently altered and plays a key role in the development of diffuse fibrillary astrocytoma is:

A. p53.

B. KRAS.

C. MDM2.

D. MDM4.

Question 25.4 Primary (de novo) glioblastoma multiforme (GBM) is commonly associated with which of the following?

A. PTEN inactivation

B. Loss of heterozygosity of chromosomes 1p and 19q

C. IDH1 mutation

D. Median age <40 years

Question 25.5 Which gene mutation identified in GBM is frequently seen in younger patients, and is associated with better prognosis:

A. EGFR.

B. p53.

C. IDH1.

D. Gain in chromosome 4.

Question 25.6 Loss of heterozygosity of chromosomes 1p and 19q is common in:

A. GBM.

B. Ependymoma.

C. Oligodendroglioma.

D. Meningioma.

Question 25.7 The central nervous system (CNS) tumor that is commonly associated with NF2 mutations is:

A. GBM.

B. Spinal ependymoma.

C. Cerebral ependymoma.

D. Oligodendroglioma.

Question 25.8 Patients with GBM have a higher likelihood of responding to therapy with epidermal growth factor receptor tyrosine kinase (EGFR-TK) inhibitors if which of the following biomarkers is present?

A. Methyl guanine methyl transferase (MGMT) gene methylation

B. Activated EGFRvIII

C. Retained PTEN function

D. B and C

Question 25.9 The primary CNS neoplasm that is associated with Epstein–Barr virus (EBV) is:

A. Primary CNS lymphoma.

B. Ependymoma.

C. Oligodendroglioma.

D. GBM.

Question 25.10 Which of the following tumors exhibit contrast enhancement on magnetic resonance imaging (MRI) scan?

A. Pilocytic astrocytoma

B. Grade 2 oligodendroglioma

C. Grade 2 astrocytoma

D. Craniopharyngioma

Question 25.11 Which of the following is associated with good prognosis in patients with low-grade gliomas?

A. Age ≥40 years

B. Tumor diameter ≥6 cm

C. Tumor crossing midline

D. Oligodendroglioma

Question 25.12 A 45-year-old man presents with generalized seizures, and MRI of the brain reveals a nonenhancing mass measuring 7 cm. Biopsy is done, and the tumor histology is reported as grade II astrocytoma. The patient undergoes surgery, and a partial (85% to 90%) tumor resection is achieved. Further treatment should include:

A. Watchful waiting.

B. Chemotherapy.

C. Radiotherapy with 50.4 Gy in 1.8 Gy fractions.

D. Radiotherapy with chemotherapy (procarbazine, CCNU, and vincristine).

Question 25.13 In patients with GBM, who have disease recurrence following initial treatment with temozolomide and radiation, the second-line treatment of choice is:

A. Erlotinib.

B. Imatinib.

C. Topotecan.

D. Bevacizumab.

Question 25.14 A 55-year-old man presents with headache and mental status changes. MRI scan of the brain reveals a 5-cm contrast-enhancing mass with surrounding edema. A stereotactic biopsy reveals GBM. The patient undergoes gross total resection of the tumor. Further treatment should include:

A. Focal external beam irradiation with 45 Gy in 30 fractions plus concurrent temozolomide.

B. Focal external beam irradiation with 60 Gy plus a 10 Gy boost plus concurrent temozolomide.

C. Focal external beam irradiation with 60 Gy in 30 fractions plus concurrent temozolomide.

D. Hyperfractionated dose of 72 Gy in 1.2 Gy fractions plus concurrent temozolomide.

Question 25.15 A 62-year-old man presents with nausea, vomiting, and severe headache. MRI scan of the brain reveals a 5-cm mass in the posterior fossa. The patient undergoes surgical resection of the mass, and pathology is reported as ependymoma. Further treatment should include:

A. Radiation therapy.

B. Concurrent chemoradiation with temozolomide.

C. Chemotherapy alone with temozolomide if MGMT gene methylated.

D. Chemotherapy alone with cisplatin plus etoposide.

Question 25.16 A 53-year-old woman presents with mental status changes; brain MRI identifies a left frontal tumor with dural marginal thickening. She undergoes a gross total resection of the tumor and the involved dural attachments. The histopathology is WHO grade I meningioma. Further treatment should include:

A. Watchful waiting.

B. Adjuvant radiation therapy.

C. Chemotherapy.

D. Radiosurgery.

Question 25.17 In immunocompetent patients with primary CNS lymphoma, with good performance status and adequate renal function, appropriate first-line therapy comprises of:

A. High-dose methotrexate.

B. R-CHOP.

C. Whole brain radiation.

D. None of the above.

Question 25.18 Long-term follow-up data showed improved survival for patients with GBM, receiving concurrent brain radiation with temozolomide compared to radiation alone, with the exception of patients with:

A. Age >50 years.

B. Unmethylated MGMT.

C. EGFR mutation positive.

D. None of the above.

Question 25.19 Which of the following is most commonly associated with craniopharyngioma?

A. Sexual dysfunction

B. Visual dysfunction

C. Hypothyroidism

D. Diabetes insipidus

Question 25.20 In adult patients diagnosed with ependymoma, adjuvant radiation therapy after resection is associated with improved overall survival in patients with:

A. Supratentorial location.

B. High-grade tumors.

C. Incomplete resection.

D. Poor performance status.

Question 25.21 In patients with anaplastic oligodendrogliomas, which gene mutation is an independent prognostic factor for improved overall survival?

A. IDH1

B. EGFR

C. p53

D. NF2

Question 25.22 Which GBM gene expression profile is most commonly associated with focal deletions or mutations in NF1?

A. Proneural

B. Neural

C. Classical

D. Mesenchymal

Question 25.23 Which three core pathways are commonly mutated in primary GBMs?

A. SHH, NF2, TRAF7

B. WNT, SHH, MAPK

C. PI3K, p53, Rb

D. NF-kB, p53, STAT

Question 25.24 Which somatic mutation is most often associated with Grade II astrocytomas?

A. CIC

B. ATRX

C. FUBP1

D. TERT promoter

Stay updated, free articles. Join our Telegram channel

Join