Space Occupying Lesions

Question 1

CNS tumour symptoms?

Answer 1

Clinical examination ; Head aches ,cognitive changes, epileptic seizures ,hemiplegia

Neurological examination papillodeama hemiplegia weakness
Associated disease carcinoma of lung , bowel ,breast ,skin lesion

CT scan single ring enhancing lesion with gadollinium contrast odema and

Biopsy to identify tumour ? Histological classification grade progress treatment

Question 2

brain tumours?

Answer 2

Primary
Glial = Astrocyte Oligodendrocytes ependymal
Neurons = embryonic tumours
Meninges =meningiomas
Secondary
Metastatic tumours

Question 3

tumour grading?

Answer 3

Histology ; therefore require a biopsy

Atypia of nuclei & pleiomorphism ( abnormal cell shape)
Vessel wall microvascular proliferation
Mitotic figures
Tumour necrosis

World Health Organization classification of CNS tumours 2007

Grading based upon histological appearances
WHO grades I II III IV

WHO I , II (LOW grades) WHO III , IV (MALIGNANT grades)

Treatment based upon this grading scheme

Question 4

symptoms of mass?

Answer 4

Local brain injury = focal neurological signs

Increased intracranial pressure = global signs
GCS falls slow vs rapid fall (Depends on tumour grade

Depends upon Rate of tumour growth
WHO grades I II ( slow growth ) or Malignant WHO III IV grades ( rapid growth )

Question 5

diffuse astrocytoma?

Answer 5

Young adults
Slow growth gradual space occupying effects
Supratentorial-

In anatomy, the supratentorial region of the brain is the area located above the tentorium cerebelli. The area of the brain below the tentorium cerebelli is the infratentorial region. The supratentorial region contains the cerebrum, while the infratentorial region contains the cerebellum.

WHO II

Survival 6-8 years
Malignant transformation into anaplastic astrocytoma about 50% at 5years

Question 6

anaplastic astrocytoma?

Answer 6

Short history rapid growth and increasing space occupying effect

Contrast enhancing on MRI
Atypical cells no necrosis usually mitotic figures
Vessel wall changes microvascular proliferation
Survival 2-3years
WHO III

Question 7

Glioblastoma?

Answer 7

Short history with neurological signs confusion ,stroke seizures
Contrast ring enhancing and odema ?
Infiltrating crosses midline
Commonest primary CNS tumour in adults

Tumour necrosis (as well as all the other histological changes ) very important finding
 WHO grade IV ; very poor survival at 18m -24m

Question 8

Pilocytic astrocytoma?

Answer 8

Children
Midline brain stem optic nerve ,cerebellum IIIventricle occasionally temporal lobe
Slow growth
MRI hyperintense on T2 can be cystic

Unlikely to have mitosis
Can spread in CSF NF1???
WHO I excellent survival at 10years

Question 9

Oligodendroglioma?

Answer 9

Young adult with seizures

Hypodense lesion in Frontal lobes OR Contrast enhancement

Oligodendroglioma WHO II
Typical cellular appearance can have calcification
Slow growth , but transforms into anaplastic variety after 3-5years
WHO II

Anaplastic Oligodendroglioma
Nuclear atypia ,multi nucleated , loose clear cytoplasm
Mitotic figures and microvascular changes
Necrosis
WHO IIII

Question 10

Ependymomas?

Answer 10

Babies ,young adults infratentorial spreads along ventricular system
Some contrast enhancement cystic blocks CSF flow

Hydrocephalus

Ependymoma
Well delineated ,pseudorossettes ,fibrillary cytoplasm of tumour cells occasional mitosis no necrosis

WHO II

Anaplastic Ependymoma
Increased atypical cellularity ,mitotic figures
WHO III

Difficult to predict biological behaviour age <3 y resection CSF spread

Question 11

embryonal tumours medulloblastoma?

Answer 11

Young children
Midline in Posterior fossa solid intensely enhancing lesion

Hydrocephalus blocks 4th ventricle

Primitive/undifferentiated neurons become malignant

CSF seeding CSF cytology

Survival 50% at 5years

Question 12

meningiomas?

Answer 12

Dural tumour

Meningioma WHO I but does infiltrate brain

Anaplastic Meningioma WHOIII
malignant cytology and recurrent +++.

Question 13

metastatic brain tumours?

Answer 13

Single or multiple sites in the CNS
From
Breast Adenocarcinoma
Bronchus Squamous cell carcinoma
Bowel Adenocarcinoma

Melanoma skin

Question 14

brain tumour therapy?

Answer 14

Await events tumour CT/MRI biopsy or operate when clinically indicated
Depends upon site ie dominant non dominant lobe

Biopsy LARGE TUMOUR and plan chemo or radiotherapy

Resection and give post operative radio therapy

Inoperable give palliative radiotherapy

Question 15

common symptoms?

Answer 15

Mechanisms of injury by a mass lesion Clinical features of raised intracranial pressure Patterns of altered focal neurological function (supra and infra-tentorial) Common causes of intracranial mass lesions Hydrocephalus

Question 16

supratentorial region?

Answer 16

The cortex (Excluding the cerebellum)

Question 17

infretentorium region?

Answer 17

The cerebelum only

Question 18

Supretentorial masses?

Answer 18

Related to Raised ICP From mass effect (tumour ± oedema) Hydrocephalus (HCP) Progressive focal neurological deficit Destruction of brain by tumour invasion Compression of brain Compression of cranial nerve Weakness → Speech (40-60% left-sided tumours) → Visual deficit →

Headache Worse in morning Associated with Valsalva Nausea & vomiting Seizures Investigate any 1st seizure > 20 yrs old Mental status change Depression, lethargy, apathy, confusion

Question 19

infretentorial symptoms?

Answer 19

Related to Raised ICP 2nd to hydrocephalus Headache Nausea & vomiting Gait disturbance Vertigo Diplopia Papilloedema

Mass effect in posterior fossa Cerebellar hemisphere → dysmetria, DDK, intention tremor Vermis → Ataxic gait, truncal ataxia Brainstem multiple cranial nerve long-tract nystagmus

Question 20

calculate the volume of the raniospinal contents?

Answer 20

v.intracranial (constant) = v.brain + v.CSF + v.blood + v.mass lesion

Question 21

patient assessment?

Answer 21

General examination Lumps Evidence of primary malignancy

Cushing’s triad - HTN (hypertension) Bradycardia Respiratory irregularity

Question 22

neurocutaneous disorders?

Answer 22

Unique cutaneous + neurological manifestations Familial tendencies Dysplasias of a number of organ systems All responsible genes have been identified 1. Neurofibromatosis 2. Tuberous Sclerosis 3. Von Hippel Lindau disease 4. Sturge Weber Syndrome 5. Ataxia-Telangiectasia 6. Osler-Rendu-Weber Syndrome

Question 23

what is neurofibromatosis?

Answer 23

Neurofibromatosis (commonly abbreviated NF; neurofibromatosis type 1 is also known as von Recklinghausen disease) is a genetically-inherited disorder in which the nerve tissue grows tumors (neurofibromas) that may be benign and may cause serious damage by compressing nerves and other tissues. The disorder affects all neural crest cells (Schwann cells, melanocytes and endoneurial fibroblasts). Cellular elements from these cell types proliferate excessively throughout the body, forming tumors; melanocytes also function abnormally in this disease, resulting in disordered skin pigmentation and café au lait spots. The tumors may cause bumps under the skin, colored spots, skeletal problems, pressure on spinal nerve roots, and other neurological problems.[1][2]

Question 24

what is tuberous sclerosis?

Answer 24

Tuberous sclerosis or tuberous sclerosis complex (TSC) is a rare multi-system genetic disease that causes non-malignant tumors to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin. A combination of symptoms may include seizures, developmental delay, behavioral problems, skin abnormalities, lung and kidney disease. TSC is caused by a mutation of either of two genes, TSC1 and TSC2, which code for the proteins hamartin and tuberin respectively. These proteins act as tumor growth suppressors, agents that regulate cell proliferation and differentiation.[1]
The name, composed of the Latin tuber (swelling) and the Greek skleros (hard), refers to the pathological finding of thick, firm and pale gyri, called “tubers,” in the brains of patients postmortem. These tubers were first described by Désiré-Magloire Bourneville in 1880; the cortical manifestations may sometimes still be known by the eponym Bourneville’s disease.

Question 25

what is von hippel lindau disease?

Answer 25

Von Hippel–Lindau (VHL) disease is a rare, autosomal dominant genetic condition that predisposes individuals to benign and malignant tumours. The most common tumours found in VHL are central nervous system and retinal hemangioblastomas, clear cell renal carcinomas, pheochromocytomas, pancreatic neuroendocrine tumours, pancreatic cysts, endolymphatic sac tumors and epididymal papillary cystadenomas.[1][2] VHL results from a mutation in the von Hippel–Lindau tumor suppressor gene on chromosome 3p25.3.[3]

Signs and symptoms associated with VHL disease include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, and high blood pressure. Conditions associated with VHL disease include angiomatosis, hemangioblastomas, pheochromocytoma, renal cell carcinoma, pancreatic cysts (pancreatic serous cystadenoma) and café au lait spots.[5] Angiomatosis occurs in 37.2% of patients presenting with VHL disease and usually occurs in the retina. As a result, loss of vision is very common. However, other organs can be affected: strokes, heart attacks, and cardiovascular disease are common additional symptoms.[3] Approximately 40% of VHL disease presents with CNS hemangioblastomas and they are present in around 60-80%. Spinal hemangioblastomas are more specific for VHL disease as 80% are found in the disease.[6][7]

Question 26

what is sturge weber syndrome?

Answer 26

Sturge–Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. It is characterized by abnormal blood vessels on the brain surface. Normally, only one side of the brain is affected.
Sturge-Weber is an embryonal developmental anomaly resulting from errors in mesodermal and ectodermal development. Unlike other neurocutaneous disorders (phakomatoses), Sturge-Weber occurs sporadically (i.e., does not have a hereditary etiology).

Question 27

what is ataxia telanhiectasia?

Answer 27

Ataxia telangiectasia (A-T) (also referred to as Louis–Bar syndrome) is a rare, neurodegenerative, inherited disease causing severe disability. Ataxia refers to poor coordination and telangiectasia to small dilated blood vessels, both of which are hallmarks of the disease.[1]
A-T affects many parts of the body:
It impairs certain areas of the brain including the cerebellum, causing difficulty with movement and coordination.
It weakens the immune system causing a predisposition to infection.
It prevents repair of broken DNA, increasing the risk of cancer.
Symptoms most often first appear in early childhood (the toddler stage) when children begin to walk. Though they usually start walking at a normal age, they wobble or sway when walking, standing still or sitting, and may appear almost as if they are drunk. In late pre-school and early school age they develop difficulty moving the eyes in a natural manner from one place to the next (oculomotor apraxia). They develop slurred or distorted speech, and swallowing problems. Some have an increased number of respiratory tract infections (ear infections, sinusitis, bronchitis, and pneumonia). Because not all children develop in the same manner or at the same rate, it may be some years before A-T is properly diagnosed. Most children with A-T have stable neurologic symptoms for the first 4–5 years of life, but begin to show increasing problems in early school years.
A-T is caused by a defect in the ATM gene,[2] which is responsible for managing the cell’s response to multiple forms of stress including double-strand breaks in DNA. In simple terms, the protein produced by the ATM gene recognizes that there is a break in DNA, recruits other proteins to fix the break, and stops the cell from making new DNA until the repair is complete.[3]

Question 28

what is osler rendu weber syndrome?

Answer 28

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu disease and Osler–Weber–Rendu syndrome, is a genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and often in organs such as the lungs, liver and brain.[1][2]
It may lead to nosebleeds, acute and chronic digestive tract bleeding, and various problems due to the involvement of other organs. Treatment focuses on reducing bleeding from blood vessel lesions, and sometimes surgery or other targeted interventions to remove arteriovenous malformations in organs. Chronic bleeding often requires iron supplements and sometimes blood transfusions. HHT is transmitted in an autosomal dominant fashion, and occurs in one in 5,000 people.[1][2]
The disease carries the names of Sir William Osler, Henri Jules Louis Marie Rendu and Frederick Parkes Weber, who described it in the late 19th and early 20th centuries.[3]

Question 29

glasgow coma scale?

Answer 29

Question 30

pupillary resonse to light?

Answer 30

Bilateral small pupils  Opiates, metabolic encephalopathies, pontine lesions Unilteral dilated pupil  expanding lesion Bilateral dilated pupils  inadequate cerebral perfusion

Question 31

karnofsky score?

Answer 31

100% - Normal, no complaints, no signs of disease
90% - Capable of normal activity, few symptoms or signs of disease
80% - Normal activity with some difficulty, some symptoms or signs
70% - Caring for self, not capable of normal activity or work
60% - Requiring some help, can take care of most personal requirements
50% - Requires help often, requires frequent medical care
40% - Disabled, requires special care and help
30% - Severely disabled, hospital admission indicated but no risk of death
20% - Very ill, requiring urgent admission, supportive measures or treatment
10% - Moribund, rapidly progressive fatal disease processes
0% - Dead

Question 32

investigations?

Answer 32

CT Head MRI Head MR Spectroscopy Functional MRI Tractography CT Chest/Abdomem

Question 33

who 2007?

Answer 33

Tumours of neuroepithelial tissue Tumours of cranial and paraspinal nerves Tumours of the meninges Lymphomas and haematopoietic neoplasms Germ cell tumours Tumours of the sellar region Metastatic tumours

Question 34

types of neuroepithelial tumours?

Answer 34

Astrocytic tumours - Pilocytic astrocytoma (grade I), Diffuse astrocytoma (grade II), Anaplastic astrocytoma (grade III), Glioblastoma (grade IV)

Oligodendroglial tumours - Oligodendroglioma, Anaplastic oligodendroglioma

Oligoastrocytic tumours

Question 35

common locations for tumours?

Answer 35

Question 36

age distribution of tumours?

Answer 36

Question 37

astrocytic brain tumours?

Answer 37

Pilocytic astrocytoma (grade I) - Children only, curable

Diffuse astrocytoma (grade II) - Slowly growing, May transform to high grade, Prognosis 2 - 20 years

Anaplastic astrocytoma/oligodendroglioma - prognosis 1 – 7 years

Glioblastoma multiforme - Prognosis 3 months - 2 years

Question 38

prognostic factors?

Answer 38

Tumour grade Age (young better, over 70 poor) Performance status (Karnofsky) Extent of surgical resection

Question 39

treatment options?

Answer 39

Surgery Radiotherapy Stereotactic radiosurgery (SRS) Chemotherapy