Pathology of CNS Tumors

CNS TumorsCNS Tumors

CPC-4.3.7 – Jenna 27y teacher. Jenna is a 27 year old teacher in Ingham who collapsed

in her classroom today. She was seen by her pupils to ‘shake all over’.

Brought to ED by paramedics, accompanied by teaching colleague. Collapsed approx 30 mins ago.

(Aim: The aim of this CPC is to get students to initially look at a broad range of differential diagnoses for a witnessed, generalized tonic- clonic seizureseizure. Then get them to focus on idiopathic epilepsyepilepsy, convulsion secondary to infection (meningitismeningitis), and convulsion secondary to brainbrain tumourtumour. get them to discuss ‘what if’ questions; outlined below are a variety of scenarios for you to draw from.

please remind students re. importance of accurate collateral history in seizure description

CPC-4.3.7 – Differential Diag. Epileptic seizure

Idiopathic - epilepsy Secondary:

Stroke - Cerebrovascular accident n.b. subarachnoid haemorrhage in this scenario.

Infection (meningitis,encephalitis) Tumour: primary or secondary Drugs: drug or alcohol withdrawal; drug overdose

cocaine; amphetamines; tricyclics, buproprion (Zyban) Genetic: neurofibromatosis;tuberous sclerosis.. Autoimmune: SLE; Hashimoto’s, encephalitis Head Injury: Trauma. Metabolic disorders: uraemia; hypoglycaemia; hypo-+

hypercalcaemia; hypo-+hypernatraemia Neurodegenerative diseases e.g. Alzheimer’s

Non epileptic events: consider syncope; cardiac arrythmias; pseudoseizures; TIA…etc

Scenario: Epilepsy: ABC breathing spontaneously rr 14/min; 4l O2 via

mask , sats (O2 Sat study) 96% ; pulse 100 bpm regular good volume T 36.1 C BP 148/94.

GCS E2V3M4 Detailed check no neck stiffness, no skin lesions/rash Tongue has been bitten; pupils equal and reactive to

light; fundoscopy normal Decreased tone R upper limb, ?normal tone other limbs Reflexes increased on R upper + lower limb; decreased

on L upper +lower; Plantar reflexes upgoing Evidence of urinary incontinence All other systems : nil abnormal Ix - BSL : 5.1; toxicology screen : negative

Scenario: Meningitis ABC breathing spontaneously rr 18/min 4l O2 via

mask, sats 90%; pulse 110 bpm reg small volume; BP 90/60 mmHg T39.6C

GCS - E2V3M4 Detailed check - petechiae non blanching rash

trunk, buttocks, Neck stiffness Small contusion L temperoparietal area Capillary refill time > 3 secs, peripheral cyanosis+ Brudzinski sign positive Ix skin scraping from lesion : gram negative

diplococci; CSF gram negative diplococci; FBC wcc 18 (polymorhic leucocytosis)

Brudzinski sign, Kernig sign, CSF findings

Core Learning Issues: Pathology Major CLI:

Raised ICP – Pathology & Clinical features. Pathology of common Primary and secondary CNS tumors in

different age groups. Astrocytoma – grades, clinical types, presentation &

complications. Pathology & Microbiology of Meningitis – common types

*Bacterial, viral, fungal & others. Pathology Minor CLI:

Pathology of Epilepsy (note this is major clinical learning issue) Meningioma, Acoustic neuroma, Craniopharyngioma / pituitary

tumors. Medulloblastoma. Overview of Pathogenesis of Epilepsy (include theories for

idiopathic epilepsy). CJD-Creutzfeldt jakob's disease. (Mad cow disease).

In every person who comes near you look for what is good and strong;

honor that; try to learn it, and your faults will drop off like dead leaves

when their time comes.

--John Ruskin

Look for good in others “No one is without faults and everyone has some good qualities…!”

Pathology of CNS TumorsCNS Tumors

Dr. Venkatesh M. Shashidhar, MDDr. Venkatesh M. Shashidhar, MDAssociate Professor & Head of Pathology

CNS Tumors: General Features

10% of all tumors. Commonest solid cancers in children.(2nd to Leuk

for all malignancies) Age: double peak 1st & 6th decade Adults - 70% supratentorial Children - 70% infratentorial No/very rare extraneural

spread. Metastasis most common.

AdultsAdults

ChildrenChildren

Clinical features:

Raised Intracranial Pressure* Headache (morning), vomiting,

slow pulse, papilloedema.Local damage:

Nerve & tract deficits, Paralysis, seizures etc.

CNS Tum: Clinical Features-Pathogenesis Headaches (morning) Papilloedema Nausea or vomiting Bradycardia Seizures (convulsions). Drowsiness, Obtundation Personality or memory Changes in speech Limb weakness Balance/Stumbling eye movements or vision

Increased ICP Increased ICP ICP – Medulla ob. ICP – Parasymp. Irritation. Brain Stem compress Frontal lobe Temporal lobe Motor area Cerebellum Optic tract, occipital.

CNS Anatomy - Clinical Features

CNS Tumors Classification:

Primary Tumors:Meninges – Meningioma

Glial cells: GliomaAstrocytoma & Glioblastoma. Oligodendroma, ependymoma.

Nerve sheath – Schwanoma, Neurofibroma.

Embryonal – Medulloblastoma, neuroblastoma, teratoma.

Blood vessels – angioma, angiosarcoma etc.

* Other Epithelial, Pituitary & Pineal gland tumors.

Secondary Tumors - Metastasis – common Melanoma, breast, lung, GIT.

Adults: Astrocytoma &

Glioblastoma. Meningioma Metastasis.

Children: Astrocytoma Medulloblastoma

Common:

Meningioma:

Arise from arachnoid granulations of venous sinuses. Attached to dura.

Common sites: parasagittal (falx), sphenoidridge, olfactory groove, cerebellopontine angle. specific clinical features.

Females common (2:1) Slow growth, well differentiated &

demarcated. Does not invade brain (Benign). Reactive skull Hyperostosis over the tumor. Microscopy: spindle cells in whorls and

psammoma bodies(microcalcification).

Meningioma

•Well demarcated•Capsulated

Meningioma – whorls of clear cells.

NormalArachnoidGranulation

Meningioma NodulesNodules

Psammoma Body

Psammoma bodies

Glioma:

Gliomas are neoplasms of glial cells. Commonest both in adults and

children Benign * to Aggressively malignant.

Astrocytoma (anaplastic & G.B.M) Ependymoma - Rare, 4th ventricle. Oligodendroglioma - Benign, adults,

Astrocytomas

Adults:Commonest 80%, Supratentorial.Solid – Fibrillary – low grade*. Varigated, Hemorrhagic - Malignant,

glioblastoma multiforme.Children:

Infratentorial (Cerebellum), Cystic, Low grade*, Pilocytic

Astrocytoma-Lowgrade fibrillary

Astrocytoma

Astrocytoma: * Lat. Vent. *petechial hem.

Glioma Brain Stem – note diffuse tumor

Glioma Cerebrum cystic degeneration

Glioma:

Astrocytoma (Glioma)

Glioma Brain Normal

Astrocytoma

Astrocytomas

Glioblastoma Multiforme (GBM): High grade Astrocytoma - Grade IV Commonest & malignant brain tumor in adults –

mean survival <1y – cerebral supratentorial. Loss of heterozygosity on Chromosome 10 (80%) Most GBMs have lost one entire copy of C – 10 2 types: Primary (worst) or Secondary from low grade

astrocytomas (better prog). Variants: giant cell GBM, gliosarcoma Microscopy: Necrosis, palisading, hypercellularity, nuclear atypia

& vascular proliferation & mitoses.

Genetic abnormalities in Glioma:Low grade Anaplastic GBM

Glioma: high grade

Glioma:Enhancement with peritumoral edema.

Glioblastoma:

GBM:+ glioma Enhancement with peritumoral edema.

Glioblastoma – high grade Astrocytoma

Glioblastoma Multiforme (high grade Astrocytoma)

Glioblastoma Cerebrum

Glioblastoma Multiforme

PalisadingPalisading

NecrosisNecrosis

NecrosisNecrosisPalisadingPalisading

Palisading

Necrosis

PalisadingNecrosis

A Astrocytoma Low gradeB Glioblastoma Multiforme(GBM)C Necrosis with pseudopalisading in GBM.

Astrocytomas

Pilocytic astrocytoma

Common in childhood Most slow growing of the gliomas Sites: cerebellum, around 3rd Ventricle. optic

nerve. Grossly cystic with mural nodule Microscopic

elongated hair-like (pilocytic) elongated cells & Rosenthal fibers.

Pilocytic Astrocytoma - children

Pilocytic astrocytomaMural nodule

Pilocytic Astrocytoma: Microscopy

Palisading pilocytic astrocytes – note plenty of Rosenthal fibres between cells.

Medulloblastoma: Children. Cerebellum – vermis. Primitive neuroectodermal tum. Blast cells – round scanty cytoplasm. 4th ventricle Obstruction – hydrocephalus. CSF seeding and Meningeal infiltration is

common. Rosettes & neuronal or glial differentiation

rarely seen.

Medulloblastoma: Primitive neuroectodermal tumor:Children, vermis of cerebellum.

Origin

Spread

Medulloblastoma

Youtube Videos: Glioblastoma Multiforme:

http://www.youtube.com/watch?v=idSos1XOi7A http://www.youtube.com/watch?v=bGawC2RJ-Sc

Meningioma: http://www.youtube.com/watch?v=ddEB5ITx2fw

Pyogenic Meningitis: http://www.youtube.com/watch?v=L9jpjxTSLws

Most common CNS Tumors:

Glioblastoma MF

CNS Tumors: Summary

Adults:Adults: Secondary commonSecondary common Lung, Skin, breast..Lung, Skin, breast.. Primary - SupratentorialPrimary - Supratentorial

Astrocytoma / Astrocytoma / glioblastoma.glioblastoma.

MeningiomaMeningioma

Children:Children: 22ndnd common (leuk / lymph) common (leuk / lymph) InfratentorialInfratentorial

Astrocytoma (cystic Astrocytoma (cystic cerebellar)cerebellar)

MedulloblastomaMedulloblastoma Hydrocephalus.Hydrocephalus. Meningeal spread.Meningeal spread.

Learning Medicine...! Learning medicine should be a JOY, not an ordeal. Everybody learns according to their own best style. The Hippocratic oath issues of patient privacy,

compassion, and FREE sharing of knowledge have to be honored.

Exam and grade anxieties are the CANCERS of medical education.

If your school admitted students which they feel need to be whipped, the SCHOOL has failed, not YOU!

If you claim you NEED to be pushed, I do not want you as my doctor.

John R. Minarcik, MD (http://www.medicalschoolpathology.com)

Pathology of Pathology of

Increased Intracranial PressureIncreased Intracranial Pressure

Pathogenesis: Increased intracranial pressure (ICP): - if >

40 mm Hg cerebral hypoxia, cerebral ischemia, cerebral edema, hydrocephalus, and brain herniation.

Cerebral edema: Edema - Disruption of the blood brain barrier – vasodilatation – swelling.

Hydrocephalus communicating type common in Total Body Irradiation.

Pathogenesis: Brain herniation: Supratentorial herniation common.

3 sub types Subfalcine herniation: The cingulate gyrus of the frontal

lobe (commonest) Central transtentorial herniation: displacement of the

basal nuclei and cerebral hemispheres downward Uncal herniation: Medial edge of the uncus and the

hippocampal gyrus Cerebellar herniation: infratentorial herniation -

tonsil of the cerebellum is pushed through the foramen magnum and compresses the medulla, leading to bradycardia and respiratory arrest.

Common CNS Herniations: Subfalcine:

Subfalcine Herniation: in brain trauma.

Contusion of the inferior temporal lobe (blue arrow) has resulted in diffuse edema. (compressed and flattened gyri on the right).

This has resulted in subfalcine herniation of the cingulate gyrus (red arrow), with a secondary hemorrhagic infarction above that (black arrow). A midline shift from right to left is also present, as is uncal herniation (yellow arrow).

Uncal Herniation:

Inferior view, The herniated uncus is bulging over the position of the tentorium (black arrows) and compressing the midbrain. The two mammillary bodies (blue arrows) have been shifted to the patients right due to the pressure.

Uncal Herniation:

acute brain swelling + Uncal Herniation

Swelling of the left cerebral hemisphere has produced a shift with herniation of the uncus of the hippocampus through the tentorium, leading to the groove seen at the white arrow.

Cerebellar Tonsil - Herniation Note the cone shape of the

herniated tonsils around the medulla in this cerebellum specimen.

Results in compression and Duret hemorrhages in the pons.

Transtentorial herniation: Transtentorial herniation

at the base of the brain. A prominent groove surrounds the displaced parahippocampal gyrus (arrow). The adjacent 3rd nerve (N) is compressed and distorted and the ipsilateral cerebral peduncle (P) is distorted with small areas of haemorrhage.

Cerebral Herniation: PathogenesisSite of Site of herniationherniation EffectEffect Clinical consequenceClinical consequenceTranstentorial Ipsilateral 3rd cranial nerve

compressionIpsilateral fixed dilated pupil

Ipsilateral 6th cranial nerve compression

Horizontal diplopia, convergent squint

Posterior cerebral artery compression

Occipital infarction Cortical blindness

Cerebral peduncle compression

Upper motor neurone signs

Brainstem compression and haemorrhage

Decerebrate posture Cardiorespiratory failureDeath

Foramen magnum

Brainstem compression and haemorrhage

Decerebrate posture Cardiorespiratory failure Death

Acute obstruction of CSF pathway

Decerebrate posture Cardiorespiratory failureDeath

Decorticate posturing, with elbows, wrists and fingers flexed, and legs extended and rotated inward.

Look for good in others… no one is without faults and everyone has some good qualities!

52y, F, CNS tumor: ? diagnosis

1 2 3 4 5

1.1. Glioblastoma m.Glioblastoma m.

2.2. AstrocytomaAstrocytoma

3.3. MetastasesMetastases

4.4. Medulloblastoma Medulloblastoma

5.5. MeningiomaMeningioma

1 2 3 4 5

6%0%0%

A.A. Glioblastoma m.Glioblastoma m.

B.B. AstrocytomaAstrocytoma

C.C. MeningiomaMeningioma

D.D. EpendymomaEpendymoma

E.E. MedulloblastomaMedulloblastoma

1 2 3 4 5

2% 2%0%0%

52y, F, parasagittal tum attached to falx: ? diagnosis

1 2 3 4 5

6% 8% 10%4%

4.4. EpendymomaEpendymoma

Commonest primary CNS tumor in Adults ?

1 2 3 4 5

52y, F, CNS tumor: ? Arrow Feature

1 2 3 4 5

0%2%0%

1.1. Necrosis.Necrosis.

2.2. Psammoma bodyPsammoma body

3.3. CalcificationCalcification

4.4. Blood vesselBlood vessel

5.5. Epithelial pearlEpithelial pearl

60y smoker, chronic bronchitis complains of difficulty walking. PE: stiff, expressionless face. A tremor of his fingers is apparent but ceases when he tries to reach for something. Image shows brain

stem . Diagnosis?

1 2 3 4 5

0%4% 2%

1.1. Alzheimers diseaseAlzheimers disease2.2. Lacunar infarctsLacunar infarcts3.3. Picks diseasePicks disease4.4. Parkinsons diseaseParkinsons disease5.5. Durett hemorrhagesDurett hemorrhages

Commonest primary CNS tumor in Children?

1 2 3 4 5

Commonest Location of CNS tumor in Children?

1 2 3 4 5

A.A. SupratentorialSupratentorial

B.B. Cerebellum Cerebellum

C.C. InfratentorialInfratentorial

D.D. Cerebrum.Cerebrum.

E.E. Brain stemBrain stem

1 2 3 4 5

46% 46%

55y Female. Died following car crash. Coroners autopsy Image shows Brain stem- What is the likely cause of death?

1 2 3 4 5

20% 20% 20%20%20%

1.1. Herniation of cerebral tonsil Herniation of cerebral tonsil

2.2. Intracerebral hemorrhage.Intracerebral hemorrhage.

3.3. Subdural hemotomaSubdural hemotoma

4.4. Subarachnoid hemorrhage.Subarachnoid hemorrhage.

5.5. Glioblastoma multiforme.Glioblastoma multiforme.

56y, F Rapidly growing parietal lobe tumor:? diagnosis

1 2 3 4 5

0%6%6%

49y, M, CNS tumor: ? diagnosis

1 2 3 4 5

A.A. MetastasesMetastases

B.B. Astrocytoma sy.Astrocytoma sy.

C.C. MeningiomatosisMeningiomatosis

D.D. NeurofibromatosisNeurofibromatosis

E.E. LipomatosisLipomatosis

54y woman dies 48 hours after suffering severe head injuries in an automobile accident. Just before her death, her left pupil becomes fixed and dilated. An inferior view of the patient's brain at autopsy is shown.

Most likely cause of death?

A. B. C. D. E.

A.A. Diffuse axonal shearingDiffuse axonal shearing

B.B. Laminar necrosisLaminar necrosis

C.C. Thrombosis of sagittal sinusThrombosis of sagittal sinus

D.D. Transtentorial herniationTranstentorial herniation

E.E. Watershed infarctWatershed infarct

1 2 3 4 5

20% 20% 20%20%20%

48y male, Frontal lobe tum, What is the most likely diagnosis?

SAQ / KFP Should seizure patients have

imaging done immediately? Personality changes indicate

which location? Differentials for young adult

with insidious symptoms, seizures and decreased signal on T1 and increased signal on T2 weighted MRI?

What is the treatment and prognosis for someone with a low-grade astrocytoma?

How should the symptoms be treated?

What tests could have been done in the absence of neuroimaging?

Yes, 10-20% tumors. Frontal lobe Other gliomas Conservative – Poor Steroids, anti

epileptic, symptomatic.

SAQ / KFP Why was the child hitting his

head? Why did the child have a

headache? If the child does have

hydrocephalus, at what level is the ventricular system being obstructed at?

Should a lumbar puncture be performed?

Where in the cerebellum is the lesion located?

What is the radiolucent area visible along the antero-superior aspect of the radiograph?

Indicating headache. Increased ICP, tum. 4th ventricle. No – coning…* Central – vermis Separation/malfusion of

anterior frontoparietal suture due to hydrocephalus.

50y Female smoker - Headache.This 50 year-old female smoker known for hypertension and diabetes mellitus type 2 was in her usual state of health until 2 years ago, when she began to have morning headaches that would usually go away by themselves. Year later began to have hearing problem on her left side. Recently, she noticed intermittent loss of sensation of the left side of her face. She is taking a thiazide diuretic, captopril, glyburide, and metformin. She has no known allergies.

Physical exam: Slight drooping in the left mouth and lower eyelid. Incomplete closure of the left eyelid with corneal touch. Reduced pain and light touch on the left side. Fundoscopic exam revealed bilateral papilledema.

50y Female smoker - Headache.

What is the most likely diagnosis?

SAQ / KFP Name the location of

tumor? What cranial nerves

are involved? List differential

diagnosis Explain pathogenesis

of headache and papilledema?

What does the histological pattern represent in slide 1? slide 2?

Cerebellopontine angle Cranial Nerves 5,7 & 8 Teratoma, meningioma,

acoustic neuroma. Increased intracranial

tension. Tumor attempting to form

Arachnoid grannulations. Origin of tumor.

35y Male, depression2-year history of loss of initiative, depression. He had slowly lost his drive to win all the big deals he always done so well at work. 3 months ago he began to experience headache, which did not respond to acetaminophen or aspirin. His wife noticed that his lethargic state had increased in the past few months. 3 days ago his right arm began to convulse uncontrollably for 1 minute. 1 day ago the patient began again violently shaking his right arm, and the right side of face began to twitch at the dinner table. No fever.

Physical exam: Bilateral papilledema, increased deep tendon reflexes of the right bicep, tricep, +ve babinski sign on the right foot, reduced leg strength on the right.

35y Male, depression

Axial T1 weighted MRI

Axial T2 weighted MRI

35y Male, depression

Coronal T1 weighted MRI

3y Male, constant cry….

Constant crying and not interacting with other children at daycare since 1m. Mother noticed that he was pointing to his head often. Family physician who stated that he was developing normally, and that the “ terrible two’s” are difficult period for parents. Recently started vomiting on a daily basis and started wobbling even though he learned to walk 6 months ago.

Physical: Bilateral papilledema and gait ataxia was noted on the physical exam.

Axial T1 weighted MRI Axial T2 weighted MRI

65y Fem morning headache.Morning headache 2y, Progressive right upper limb weakness. She woke up this morning obtunded, and did not initially respond to her husband’s cries. She screamed to her husband that she could not see anything to her right, and that she that her left arm and leg were very weak. At this point her husband rushed her to the nearest hospital.Physical Exam: left lid ptosis, left-pupillary dilation, and failure of her left eye to constrict to light directly or consenually. Patient had bilateral lower limb weakness, with increased deep tendon reflexes on the left side, and a +ve babinski on the left side. Bilateral Papilledema. Homonymous hemianopia of the right side. Visual acuity was corrected to 20/20 with glasses.

65y Fem morning headache.

Brain Metastasis: Lung, Breast, Skin,

Kidney, GIT. Prostate – never..! Well demarcated,

usually multiple with surrounding rim of inflammation.

Carcinomatosis: Meningeal CSF spread of malignant cells.

Metastatic Melanoma: multiple

Brain Metastases: Surrounding edema.

SAQ / KFP Are there clinical signs of

nerve compression? What is the most likely cause

of the homonymous hemianopia?

Why does the patient have progressive right upper limb weakness, and paroxysmal left upper and lower limb weakness?

Should a lumbar puncture be performed?

Why was the patient obtunded?

Why was an-x-ray taken?

Yes, ptosis, pupils 3rd Optic pathway -

occipital. Motor cortex

compression – tum. Risky. Brainstem

compression. Meningioma

hyperostosis.

CPC-3.7– KFP Questions: Meningitis Types, classification & comparison. Septic, Viral & TB meningitis. Morphology, complications. Laboratory diagnosis, CSF findings. CNS tumours: common features. Adult and childhood CNS tumors. Common Types & features. Increased intracranial pressure – Pathologic

basis of clinical features.

It has been my philosophy of life that difficulties vanish

when faced boldly.

--Isaac Asimov

Other CNS tumors

Neuroectodermal Tumors

Origin from primitive blast cells. Rosettes - attempted nerve formation.

1. Medulloblastoma – Cerebellum

2. Retinoblastoma - Retina

3. Neuroblastoma – Adrenal glands

4. Ganglioneuroma - Mediastinum

Medulloblastoma

Ependymoma-hemorrhage

Ependymoma 4th Ventricle

Ependymoma

Nerve Sheath Tumors:

Neurofibroma: Epi & endoneurial fibroblasts. Form whorls of fibroblasts with nerves Well differentiated, benign, capsulated.

Schwannoma: Schwann cells, elongated form whorls Nuclear palisading

Schwannoma / Neurofibroma

Schwannoma 8th Nerve:

Bilateral 8th nerve schwannomas.

Schwannoma:

Schwannoma

Neurofibromatosis:

Café-au-lait spot

Schwannoma

Summary: Children – 70% INFRAtentorial Adults – 70% SUPRAtentorial Common Malignant - adults, metastatic tumors (Lungs) Common - adults – glioblastoma multiforme

Intracerebral Common Benign - children – cerebellar astrocytoma. Common Mal - children – cerebellar medulloblastoma Very rare – meninges and schwann cells (meningiomas

and schwannomas) – usu. found in adults

A 26-year old femaleHeadache,vomiting, an epileptic attack, weakness of legs. Now drowsy. Two weeks before admission she gave her second birth.

CT and NMR revealed a huge parasagittal tumor (80x67x65 mm), enhanced by contrast, compressed corpus callosum and ventricles.

Histopathology:

Bifrontal parasagittal tumor, craniectomy and tumor was totally removed.

Well demarcated, firm white lobular.

Fibrous – spindle cells.

37 yr FemaleSerious automobile accident and sustained a close head injury,she does not immediately seek medical attention, but is brought to the emergency room two hours later by her brother,on physical examination there is mydriasis and loss of pupillary light reflex,several hours later she is unable to follow a flash light with her eyes,which of the following herniation is most likely occuring in this patient????

A)cerebellar tonsils into the forman magnum B)cerebellum upward past the tentorium C)singulate gyrus under the falx D)medulla into the foramen magnum E)temporal lobe under the tentorium

32y Female Fleshy pappules: Several fleshy papules

on face, trunk, and upper extremities.

Since 10y of age. Increased & Irritation over the past 5 y.

Previous excision have recurred.

No other significant history.

Neurofibromatosis: Autosomal dominant, NF1- Peripheral/Von Recklinghausen’s NF2- known as central NF. However, NF1 may cause central characteristics. About 50% familial, 50% sporadic gene mutation. NF1/ von Recklinghausen disease, gene mutation on

chromosome 17, 1 in every 3000-4000 births. Diagnosis of NF1 if > 2 of 6 or more café au lait spots (irregularly shaped, evenly

pigmented, brown macules), 2 or more neurofibromas, axillary or inguinal freckling, Lisch nodules on the iris or optic glioma, various types of osseous lesions, a first-degree relative with the condition.

Neurofibromatosis: NF2 – Gene mutation chromosome 22. 1 in every 33,000-40,000 births Typically present with acoustic neuromas or vestibular

schwannomas. Tinnitus, balance disorders, and progressive hearing loss May also have meningiomas and juvenile cataracts. First-degree relative and on any 2 of the conditions listed for NF1. Patients with NF1 are at increased risk of malignancy. Annual ocular examinations are recommended. Genetic testing is

also advocated in patients with NF who wish to have children. Surgery has been a successful treatment for the lesions

themselves; however, recurrence often occurs, and nerve damage is a risk when tumors are located along neural pathways

(National Institute of Neurologic Disorders and Stroke, 2006).

7th nerve palsy: Cerebellopontine angle

tumours. Acoustic neuroma,epidermoid cysts, medulloblastomameningioma

Affected cranial nerves:5 trigeminal - masticatio7 facial –face muscles8 auditory – hearing

Brudzinski Sign of Meningitis:

Scenario: Brain Tumor ABC as for scenario 1 GCS E3V4M5 Detailed check no neck stiffness, no rash Tongue has been bitten; small contusion L

temperoparietal area PEARL fundoscopy normal L sided weakness arm >

leg with increased tone and reflexes L plantar reflex equivocal; R plantar reflex downgoing

Evidence urinary incontinence All other systems : no abnormalities Ix - as per scenario 1; MRI scan ?gliobalstoma multiforme R fronto-parietal region

GBM: Glomeruloid bodies:

Normal Fundus - Papilledema

Normal vs Glaucoma

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