Tumours of the nervous system Flashcards

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1
Q

How common are brain cancers in children?

A

The second most common cancer in children

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2
Q

What is Basal Naevus Syndrome (Gorlin Syndrome)?

A

Rare genetic condition affecting the skin, endocrine system, nervous system, eyes and bones.
Associated with the development of basal cell carcinoma in adolescence or young adulthood.

Other cancers associated with the syndrome are medulloblastoma, breast cancer, non-Hodgkin’s lymphoma and ovarian cancer.

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3
Q

What is Li Fraumeni Syndrome?

A

Soft tissue sarcoma - cancers associated with this syndrome are brain tumours, breast cancer leukaemias, and adrenocortical cancer.

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4
Q

What is the leading cause of death in children under the age of 15 years old?

A

Brain tumours

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5
Q

How many children died from a brain or other tumour in 2015-2017? CRUK

A

236

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6
Q

What percentage of primary malignant lesions are CNS tumours?

A

9%

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7
Q

What is the aetiology of brain tumours?

A

Unknown

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8
Q

What are possible carcinogens?

A

Radiotherapy to the head (ionising radiation) – always chance of secondary malignancy

Hairdressers and those using hair dye have been claimed to be at greater risk

Chemicals used in the rubber industry

Head injury

History of previous exposure to toxoplasma bacteria

Neurofibromatosis or Turcot’s syndrome

Swedish study found those in the higher socioeconomic groups were greater risk of glioma

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9
Q

What is Von Hippel Lindau Syndrome?

A

A protein which causes certain cells to behave abnormally and appear to be starved of oxygen. The result of this is that blood vessels grow around the affected cells which can develop into benign tumours or cysts (sometimes malignant)
ACE Angiotensin converting enzyme – causes hypertension

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10
Q

What is the percentage of developing Von Hippel Lindau Syndrome is one of your parents has it?

A

50%

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11
Q

What is Phaechromocytoma?

A

Tumour of the adrenal medulla which produce ACE Angiotensin converting enzyme

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12
Q

What is Turcot syndrome?

A

When colon polyposis or adenocarcinoma are diagnosed with medulloblastoma or glioblastoma the patient can be suspected as having Turcot syndrome

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13
Q

When can Turcot syndrome develop?

A

1st/2nd decade of life

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14
Q

What is Tuberous Sclerosis?

A

Raised papules in the skin which can resemble acne

TSC may cause tumours to form in various organs. In addition to the skin, growths may occur in the brain, eyes, heart, kidney, and lungs

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15
Q

Tumour - Benign or Malignant - Treatment (Neurological tissue)

A

Glioma - Malignant - Grade I-II Surgery

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16
Q

Tumour - Benign or Malignant - Treatment (Astrocytes)

A

Astrocytoma - Malignant - Grade I-II Surgery

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17
Q

Tumour - Benign or Malignant - Treatment (Oligodendrocytes)

A

Oligodendroglioma - Malignant - Grade III-IV Radiotherapy
Radiotherapy (surgery)

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18
Q

Tumour - Benign or Malignant - Treatment (Glio…)

A

Glioblastoma - Malignant - Grade III-IV Radiotherapy
Radiotherapy (surgery)

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19
Q

Tumour - Benign or Malignant - Treatment (Ependymal Cells)

A

Ependymoma - Benign - Surgery (radiotherapy)

Ependymoblastoma - Malignant - Radiotherapy

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20
Q

Tumour - Benign or Malignant - Treatment (Sheath of Nerve Cells)

A

Neurofibroma - Benign - Surgery

Neurolemmoma - Malignant - Surgery (radiotherapy)

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21
Q

Tumour - Benign or Malignant - Treatment (Meninges)

A

Meingioma - Benign - Surgery (radiotherapy)

Menigiosarcoma - Malignant - Surgery

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22
Q

Tumour - Benign or Malignant - Treatment (Vascular tissue)

A

Angioma - Benign - Surgery

Arterio-venous Malformation(AVM) - Benign - Surgery (radiotherapy)

Haemangioblatoma - Malignant - Surgery

23
Q

Grade 1

A

Myxo-papillary ependymoma and sub-ependymoma

24
Q

Grade 2

A

Ependymoma (low-grade)

25
Q

Grade 3

A

Anaplastic (malignant) ependymoma.

26
Q

Tumour - Benign or Malignant - Treatment (Embryonic tissue)

A

Pinealoma - Benign - Surgery

Craniopharyngioma - Benign - Surgery (radiotherapy)

Teratoma / Seminoma - Malignant - Surgery (chemotherapy)

Medulloblastoma - Malignant - Surgery

27
Q

Tumour - Benign or Malignant - Treatment (Reticular tissue)

A

Microglioma - Malignant - Radiotherapy

Lymphoma - Malignant - Surgery and radiotherapy

28
Q

Tumour - Benign or Malignant - Treatment (Pituitary)

A

Adenoma - Benign - Surgery and radiotherapy

Adenocarcinoma - Malignant - Surgery and radiotherapy

29
Q

Tumour - Benign or Malignant - Treatment (Metastatic tumours)

A

Lung, breast, kidney GI melanoma - Malignant - Surgery, radiotherapy, chemotherapy

30
Q

Where do childhood tumours most commonly arise?

A

Posterior fossa

31
Q

Where do medulloblastomas occur?

A

4th ventricle, thought to initiate from primitive cerebellar cells

32
Q

Where do ependymomas occur?

A

ependymal cells of the ventricles and central canal of spinal cord

33
Q

3 most common childhood tumours?

A

Medulloblastomas

Ependymomas

Cerebellar astrocytoma

34
Q

Where do adult tumours most commonly arise?

A

Cerebral hemispheres > mainly frontal area

35
Q

What is the most common adult tumour?

A

Gliomas

36
Q

What percentage of ependymomas are myxopapillary ependymoma and benign?

A

85%

37
Q

What are 4 relatively benign tumours of adults?

A

Intracranial menigioma

Neurolemmoma

Acoustic neuroma

Pituitary adenoma

38
Q

Who are intercranial menigiomas more common in?

A

Tumour of middle age
More common in women

39
Q

What are metastases

A

Secondary deposits which have spread from primary tumours arising in other sites e.g. breast, lung, kidneys and testes.

They form a significant number of the CNS tumours treated in the RT department

40
Q

What is Raised Intercranial Pressure (ICP)?

A

Space occupying lesions will eventually cause raised ICP. Initially the ventricles may be displaced. As the tumour increases in size the flow of CSF becomes obstructed

41
Q

Where to primary brain tumours rarely metastasise outside of?

A

The nervous system

42
Q

Signs and symptoms

A

Headache (Dull intolerable pain - delirium, May develop over long period, Can be acute, Often described as being more intense in the frontal region and more severe in the morning, Delirium may be due to the severity of the pain caused by raised ICP; there is intense pressure on the brain. May be due to brain destruction by tumour.)

Vomiting (Due to raised ICP
Projectile (characteristic of medulloblastoma), Pressure on the vomiting centre in the brain stem.
Pain from the headache, Typically projectile vomiting is seen in children with medulloblastoma although any patient with a space occupying lesion can experience this. Some patients never experience headache)

Epilepsy (Sudden onset, No apparent cause, No recorded history, May herald the presence of a brain tumour)

Visual disturbances (Diplopia
Homonymous hemianopia
Tunnel vision)

Balance and Gait disturbances (May find it difficult to stand or walk
Walks with an splayed gait)

43
Q

Signs and symptoms of Peripheral Neuropathy

A

The patient may describe strange sensation in the arms legs fingers and toes

44
Q

Signs and symptoms of Beaten Copper

A

In children where the bone of the skull has not ossified an x-ray may display a beaten copper appearance due to the convolutions of the brain being pressed against the inner skull.

45
Q

Signs and symptoms of Suture

A

Separation of the sutures may be seen in children

46
Q

What can slow growing lesions cause?

A

Astonishing brain displacement without causing neurological disturbance.

47
Q

What can rapid growing lesions cause?

A

Dramatic signs and symptoms causing the patient to deteriorate suddenly. Unless the pressure is promptly relieved the patient will die.

48
Q

Diagnosis

A

Full case history

Chest x-ray

FBC

Clinical examination (Fundoscopic examination
Visual field
Olfactory and taste test)

49
Q

The World Health Organisation (WHO) classify a tumour by…

A

Tissue of origin (neuroepthelium, meningial)
Cell type (astrocyte, oligodendrocyte)

50
Q

Grading

A

Takes account of the degree of differentiation and the clinical behaviour of a tumour

51
Q

TNM Classification

A

Not used as primary cerebral tumours rarely metastasize out with the CNS

52
Q

Why does a tumour which displays rapid growth patterns is likely to be fatal why?

A

Because of the gross neurological damage it can do in a very short period and the increased chance of herniation.

53
Q
A