CNS Malignancies FRCR CO2A

Question 1

What anatomical areas are covered in the management of CNS tumors?

Answer 1

Cerebral convexity and hemispheres, skull base, pituitary, pineal region, and spinal cord.

Question 2

Why are the terms malignant and benign not very useful for CNS tumors?

Answer 2

Small slowly growing tumors can cause severe symptoms; surgery can be difficult due to infiltration; most tumors rarely metastasize outside the CNS; slow-growing tumors may transform into aggressive variants.

Question 3

What does this chapter not cover regarding CNS tumors?

Answer 3

Metastatic disease to the CNS, although management of cerebral metastases requiring specialist neuro-oncology input is briefly discussed.

Question 4

What anatomical structure separates the supratentorial from the infratentorial areas of the brain?

Answer 4

The tentorium.

Question 5

What is Broca’s area responsible for?

Answer 5

Expressive speech.

Question 6

What is Wernicke’s area responsible for?

Answer 6

Receptive speech.

Question 7

What is the incidence of primary brain and CNS tumors?

Answer 7

Approximately 15 in 100,000 per annum.

Question 8

How many primary tumors of the brain and CNS were registered in the UK in 2011?

Answer 8

9365 primary tumors.

Question 9

What percentage of all cancers do brain tumors account for in the UK?

Answer 9

3%.

Question 10

What is a significant risk factor for developing primary CNS tumors?

Answer 10

Increasing age, male gender, and higher socioeconomic status.

Question 11

What is a well-recognized cause of cerebral lymphoma?

Answer 11

Acquired immune deficiency syndrome (AIDS).

Question 12

What are some inherited cancer syndromes associated with CNS tumors?

Answer 12

Neurofibromatosis types 1 and 2, von Hippel–Lindau syndrome, tuberous sclerosis, Li–Fraumeni syndrome, Cowden’s disease, Turcot’s syndrome, and naevoid basal cell carcinoma syndrome.

Question 13

What is the classification of CNS tumors based on the WHO?

Answer 13

WHO grade I includes low proliferative potential tumors; grade II includes infiltrating tumors; grade III includes tumors with histological evidence of malignancy; grade IV includes mitotically active and necrosis-prone tumors.

Question 14

What is the most common type of pituitary tumor?

Answer 14

Adenomas (95% of pituitary tumors).

Question 15

What are the three main histological types of tumors involving the pineal gland?

Answer 15

Germ-cell tumors, astrocytomas, and pineal parenchymal tumors.

Question 16

What is the role of molecular markers in neuro-oncology?

Answer 16

They help predict prognosis and determine treatment.

Question 17

What are the main modes of spread for CNS tumors?

Answer 17

Local invasion, local pressure, CSF spread, and rare haematogenous spread.

Question 18

What are common symptoms of primary CNS tumors?

Answer 18

Headaches, seizures, changes in mental state, unilateral deafness, and progressive neurological deficits.

Question 19

What is a key principle of management for CNS tumors?

Answer 19

All patients must be discussed at a neurosciences multidisciplinary team (MDT) meeting.

Question 20

What key investigations should be coordinated by a neuro-oncology MDT?

Answer 20

Routine blood tests, imaging, angiography, and biopsy.

Question 21

What imaging technique is preferred for initial investigation of CNS tumors?

Answer 21

MRI.

Question 22

What are some methods for obtaining tissue for diagnosis?

Answer 22

Stereotactically guided biopsy, radiologically guided needle biopsy, and endoscopic biopsy.

Question 23

What is the importance of intraoperative histopathological diagnosis?

Answer 23

It helps ensure enough of the right tissue is obtained and can influence the course of the operation.

Question 24

What additional assessments may be required for patients with skull base tumors?

Answer 24

Audiological testing, auditory-evoked brain stem responses testing, vestibular testing, and prosthetic assessment.

Question 25

What are PECT and PET primarily used for?

Answer 25

PECT and positron emission tomography (PET) are currently predominantly research tools and are not in day-to-day use.

Question 26

Which patients are at particular risk of developing intradural spinal tumours?

Answer 26

Patients with neurofibromatosis types 1 and 2 are at particular risk and require monitoring and early resection if tumours enlarge or cause symptoms.

Question 27

What assessments may be required for patients with skull base tumours before treatment?

Answer 27

Patients may require audiological testing, auditory-evoked brain stem responses testing, vestibular testing, prosthetic assessment, speech and language therapy assessment, and dietetic assessment.

Question 28

What is the status of TNM staging for brain tumours?

Answer 28

TNM staging for brain tumours has been withdrawn and does not contribute to the management of most primary CNS tumours.

Question 29

What staging determines treatment protocols in medulloblastoma?

Answer 29

Chang staging determines treatment protocols in medulloblastoma.

Question 30

What percentage of adult brain tumours are low-grade gliomas (LGGs)?

Answer 30

Low-grade gliomas account for approximately 15% of all adult brain tumours.

Question 31

What is the initial management for low-grade gliomas?

Answer 31

The options for initial management are either watchful waiting or immediate surgery.

Question 32

When is radiotherapy used for low-grade gliomas?

Answer 32

Radiotherapy may be used for patients with persisting neurological symptoms, significant residual tumour, regrowth following surgery, or evidence of tumour progression from low to high grade.

Question 33

What did the EORTC 22033–26033 trial report regarding treatment options?

Answer 33

The trial showed no survival difference between patients treated with radiotherapy or temozolomide.

Question 34

What are high-grade gliomas (HGGs)?

Answer 34

High-grade gliomas include glioblastoma, anaplastic astrocytoma, anaplastic oligodendrogliomas, and anaplastic ependymomas.

Question 35

What are important prognostic factors for high-grade gliomas?

Answer 35

Important prognostic factors include age, performance status, comorbidity, tumour type and grade, and presence or absence of seizures.

Question 36

What is the goal of treating high-grade gliomas?

Answer 36

The goal is to increase survival while maximizing the patient's functional capability and quality of life.

Question 37

What is the standard of care for good performance status patients with grade IV astrocytomas?

Answer 37

Combined temozolomide and radiotherapy is the standard of care.

Question 38

What has been shown to improve survival in patients with newly diagnosed high-grade gliomas?

Answer 38

Carmustine implants in combination with surgical resection and adjuvant postoperative temozolomide concurrently with radiotherapy have been shown to improve survival by approximately 2–3 months.