DR.G .BALAJIPROF.DR.G.SUNDARAMURTHY’S unit

History 35 year old male presented with seizures- 4

episodes.GTCS , involving all 4 limbs.Each episode lasting for about 5 mins. Pt was

unconscious in the inter ictal period.h/o tongue bite, frothing present.h/o bladder incontinence during the seizures.No h/o fever, headache, vomiting, alterred

sensorium.No h/o trauma, head injury, blurring of vision, ent

bleed.

Past history:Not a known case of DM/SHT/IHD/PT/BA.h/o 1 episode of seizures 3 months back. Treated

at a private hospital. Details not known.No h/o seizures in childhood.Pt underwent appendicectomy at vhs hospital.Personal history: occasional alcohol consumer.Bowel, bladder habits are normal. No h/o of

addictions, premarital or extramarital sexual affairs

On examinationPt was conscious, drowsy , afebrileHydration- fairPulse- 88/ min, regular, normal volumeBp: 110/76 mm hg in rt ul in supine positionNo pallor/icterus/ cyanosis/ clubbing/ pedal

edema/ cyanosis/ lymph adenopathy.

Cvs- s1,s2 heard.No murmursRs- NVBS heard, no added soundsPer abdomen- soft , non tender.No organomegaly, no free fluid.Cns- no focal neurological deficit.PLANTAR-B/L FLEXORFundus- no pappilloedema

Provisional diagnosis SEIZURE DISORDER- LATE ONSET ? CAUSE

Investigations:CBCTC-6500 cell/mm^3DC: p-78, l-22.Hb-12.8 gramsPlatelets-2.4 lakhs.ESR: 4/10 mmRFT: urea- 30 mg, creatinine-0.8 mgUrine routine: albumin-trace, sugar- nilDeposits- 1-2 pus cells

Patient was treated with iv diazepam, phenytoin.

CT brain was taken on 27/8/10Showed an ill defined hypodense lesion in rt

frontal lobe- suspected ischemic infarct.Neurologist opinion- ?mass in right frontal lobe, suggested MRI

brain

MRI brain:[02/09/10]Intra axial lesion predominantly involving

white matter, in right frontal lobe with effacement of lateral ventricles, herniation of cingulate sulcus, compression of corpus collosum, with midline shift. The lesion is hetero intense in T1, T2 Weighted images. No diffusion restriction seen.

Features suggestive of anaplastic astrocytoma.

NEUOSURGEON’S OPINION:ANAPLSTIC ASTROCYTOMA/ GLIOMA-low

gradePt was advised surgeryPt was not willing for surgery at Stanley .

Discharged at request.

On 13/10/10 Patient underwent surgery at VHS hospital. Patient underwent right frontal craniotomy with radical excision of tumour.

post op period was uneventful. Patient undergoing radiotherapy at VHS

hospital.

HPE- uniform sheets of compactly arranged round cells, separated in to lobules, and interspersed by micro cystic spaces. Round cells shows uniform nuclei.

Imp: low grade oligodendroglioma

HPE at NIMHANS- Grade III anaplsatic oligodendroglioma

Post operative periodCT brain-[19/10/10]Residual cystic mass in rt fronto temporal

region with mass effect and midline shift.Post op changes with extra axial hge in rt

fronto temporal region.CT brain[10/11/10]Residual cystic lesion in rt frontal lobe. Size

decreased compared to previous ct.Pt currently on radiotherapy

T

CNS TUMOURS

WHO CLASSIFICATION

Neuroepithelial Tumor

GliomaAstrocytomasOligodendrogliomasEpendymomaChoroid Plexus Tumor

Pineal Tumor

Neuronal TumorGangliogliomaGangliocytomaNeuroblastoma

Medulloblastoma

Nerve Sheath Tumor Vestibular Schwanoma

Meningeal Tumor Meningioma

Pituitary Tumor

Germ Cell Tumor Germinoma

Lymphomas Teratoma

Tumor Like Malformation

CraniopharyngiomaEpidermoid TumorDermoid TumorColloid Cyst

Metastatic Tumor

Contiguous extension from regional Tumor ( Glomus Tumor )

Genetic LinkingDisease Mutation Protein Tumour

Neurofibromatosis Type 1

Chromosone 17

Nourofibromin

AstrocytomaNeuroma,

schwannoma, optic glioma

Neurofibromatosis Type 2

Chromosone 22

Schwannomin Acoustic neuroma,

gliomaMeningioma,

Li-Fraumeni’sSyndrome

Chromosone 17

P 53 Malignant glioma

Basal cell naevus syndrome

PTCH gene Medulloblastoma

Familial Adenomatous

Polyposis Syndrome

APC gene Medulloblastoma, malignant

glioma

syndrome Gene locus Gene product Neoplasms in NS

TUBEROUS SCLEROSIS

TSC1[9q]TSC2[16P]

HamartinTuberin

Astrocytoma

VON-HIPPEL- LINDAU

VHL[3p] pVHL Hemangioblastoma of retina, cerebellum , spinal cord.

retinoblastoma RB1[13q] RB Retinoblastoama, pineoblastoma, malignant glioma

MEN1[Werner syndrome]

MEN1[11q 13] menin Pituitary adenoma, malignant schwannoma

GLIOMAa type of tumor that starts in the brain or spine. It is called a glioma because it arises from glial cellsThe most common site of gliomas is the brain

ClassificationClassified - by cell type, - by grade, - by location.

By gradeGrade Differentiation Type Prognosis

Low-grade well-differentiated (not anaplastic)

benign better

High-grade undifferentiated (anaplastic)

malignant worse

HISTOLOGICAL CRITERIA

Cellularity

Nuclear pleomorphism

Mitoses

Vascular proliferation

Necrosis

GLIOMA

WHO CLASSIFICATION

Astrocytomas

Oligodendrogliomas

Ependymoma

Choroid Plexus Tumor

GRADING

GRADE I Pilocytic Astrocytoma

GRADE II Diffuse Astrocytoma

GRADE III Anaplastic Astrocytoma

GRADE IV Glioblastoma Multiforme

By locationType Location Site Age

Supratentorial above the tentorium

in the cerebrum

mostly in adults (70%)

Infratentorial below the tentorium

in the cerebellum

mostly in children (70%)

By cell type

Glial Cells Glial Tumour

Astrocytes Astrocytomas

Oligodendrocytes Oligodendrogliomas

Ependymal cells Ependymomas

Different types of glia Mixed gliomas (oligoastrocytomas)

PrognosisGliomas cannot be cured

High-grade gliomas Poor prognosis50% - 1 year after diagnosis25% - 2 years after diagnosisanaplastic astrocytoma survive about three yearsGlioblastoma multiforme has a worse prognosis

PROGNOSIS

Histological grade

Cell type

Tumor size

Patient’s factor

SymptomsDepends on which part of the central nervous system is

affected

Drop metastases not metastasize by the bloodstream

but they can spread via the cerebrospinal fluid and cause "drop metastases" to the spinal cord

Affected organ Symptoms

brain glioma increased intracranial pressureheadaches, nausea, vomiting, seizures,

cranial nerve disorders

optic nerve glioma visual loss

Spinal cord gliomas pain, weakness, numbness in the extremities

PathologyHigh-grade gliomas highly-vascular tumorstendency to infiltrateextensive areas of necrosis and hypoxiabreakdown of the blood-brain barrieralmost always recur even after complete surgical excision

Low-grade gliomas grow slowlyover many yearsfollowed without treatment unless they grow and cause symptoms

Treatment: surgery radiotherapy chemptherapySURGERY: for obtaining specimen for hp diagnosis, debulking for removing the mass effect.Extent of tumour removal correlates with

survival in young.

RADIOTHERAPY:Post op radiotherapy imprvoves survival, quality of

life.In primary glial tumours given to tumour mass

with a 2cm margin.Total dose- 5000 to 7000 cGy given in 25- 35 equal

fractions, 5 days a week.CHEMOTHERAPY:Adjuvant to surgery and radiotherapy.Temozolamide- orally acting alkylating agent.

Better tolerated than nitrosureas. Most widely used agent for high grade gliomas

GLIOMATOSIS CEREBRIDiffuse infiltration of brain by malignant

astrocytes without a focal enhancing mass.Presents as a multifocal cns syndrome or as a

genralised disorder with dementia, seizures and personality changes.

Imaging not helpful in diagnosis. Biopsy confirms the diagnosis

Treated with whole brain irradiation or temozolomide.

Karanofsky performance index100- normal, no complaints, no evidence of

disease.90- able to carry normal activity, minor signs

or symptoms of disease.80-normal aactivity with effort, some signs or

symptoms of disease.70-care for self. Unable to carry on normal

activity or do active work.60-require occasional assistance. But able to

care for most needs

50-requires considerable assistance and frequent medical care.

40- disabled. Requirees special care and assistance

30-severely disabled. Hospitalisation is indiacated.

20-very sick. Hospitalisation is necessary. Needs active support.

10- moribund.0- dead

Oligodendroglioma

Incurableslowly growing with prolonged survivalmedian survival times -7-8 years for grade II -3.5 years for grade IIIvery high (almost uniform) rate of recurrence gradually increase in grade over time

Tumour Grading

Oligodendroglioma II

Anaplastic oligodendroglioma III

Oligodendrogliomaa type of glioma that are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell.

derives from the Greek roots 'oligo' meaning “ few” 'dendro' meaning “trees”

They occur primarily in adults 15% of gliomas in adults.Slow growing, more responsive to cytotoxic agents.Supratentorial location.mitosis., atypia, necrosis are associated with an aggressive course.

Symptoms

first symptom - seizure frontal lobe - affecting personalityHeadaches combined with increased intracranial pressureDepending on the location of the tumor, any neurological deficit

(CT) or (MRI) scan is necessary to characterize the anatomy of this tumor (size, location, heter/homogeneity)

final diagnosis of this tumor, relies on histopathologic examination (biopsy examination).

Microscopic Appearance

characteristic fried egg-like cells, with clear cytoplasm and well-defined cell borders. H&E stain.

composed of cells with small to slightly enlarged round nucleiwith dark, compact nuclei and a small amount of eosinophilic cytoplasm

Micrograph of an oligodendroglioma

showing the characteristic branching, small,

chicken wire-like blood vessels and fried egg-like cells,

with clear cytoplasm and well-defined cell borders.

H&E stain.

Copyright © American Society of Clinical Oncology

Brandes, A. A. et al. J Clin Oncol; 24:4746-4753 2006

Fluorescence in situ hybridization analysis of anaplastic oligodendroglioma displaying deletion on 1p

Oligodendroglioma• 1p/19q• Good outcome• Chemosensitivity

TREATMENTChemotherapy TEMOZOLOMIDECombination chemotherapy with PROCARBAZINE, LOMUSTINE and VINCRISTINE.Oligodendrogliomas with deletion of 1p and 19q predicts a durable response to chemotherapy.SurgeryBecause of their diffusely infiltrating nature, oligodendrogliomas cannot be completely resected not curable by surgical excisionSurgery may be followed up by chemotherapy, radiationStereotactic surgery small tumors that have been diagnosed early

ASTROCYTOMAClassificatio

nTumour Grade

I Pilocytic astrocytoma I Low

I A Pilomyxoid astrocytoma II Low

II Subependymal giant cell astrocytoma

I Low

III Pleomorphic xantho astrocytoma II Low

IV Diffuse astrocytoma II Low

V Anaplastic astrocytoma III High

VI Glioblastoma IV High

VI A Giant cell glioblastoma IV High

VI B Gliosarcoma IV High

VII Gliomatosis cerebri III High

ASTROCYTOMA

Astrocytomas are cancers of the brain that originate in star-shaped brain cells called astrocytesmost common primary CNS malignancy75% of neuroepithelial tumors

low grade - < 6 % of Intracranial tumorsgrade (IV) - 20 % of Intracranial tumors - survival 3 - 12 months

Glioblastoma multiforme

standard name - “Glioblastoma”- most common - most aggressive - 52% of all parenchymal brain tumor- 20% of all intracranial tumorsClassified - Giant cell glioblastoma - Gliosarcoma

Glioblastoma multiformeCauses

Sporadic , without any genetic predispositionNo links - smoking,diet, cellular phones, electromagnetic fieldsSex: male Age: over 50 years old Ethnicity: Caucasians, Latinos, Asians Linked - low-grade astrocytoma (brain tumor) - genetic disorder Neurofibromatosis, Tuberous sclerosis, Von Hippel-Lindau disease, Li-Fraumeni syndrome, Turcot syndrome - cytomegalovirus. - ionizing radiation - polyvinyl chloride

Glioblastoma multiforme

Symptomsdepends highly on the location asymptomatic conditionseizure, nausea and vomiting, headache, and hemiparesisprogressive memory, personality, or neurological deficit due to temporal and frontal lobe involvement

DiagnosisMRI ring-enhancing lesionsDefinitive diagnosis of a suspected GBM on CT or MRI stereotactic biopsy or craniotomy with tumor resection

Glioblastoma multiforme

Sagittal MRI with contrast glioblastoma

WHO grade IV in a 15-year-old boy

Glioblastoma multiformePathogenesispresence of small areas of necrotizing tissue that is surrounded by anaplastic cells (pseudopalisading necrosis)differentiates Grade 3 astrocytomas presence of hyperplastic blood vesselssecondary GBM degeneration of lower grade gliomas more common in younger patientsIn the cerebral white matter, grow quicklyextend into the meninges or ventricular wall high protein content in the (CSF) (> 100 mg/dL)About 50% of GBM > one lobe of a hemisphere or are bilateralclassic infiltration across the corpus callosum butterfly (bilateral) gliomaMass effect - tumor and edema compress the ventricles and cause hydrocephalus

Glioblastoma multiforme

Treatment very difficultThe tumor cells are very resistant to chemotherapy and other conventional therapies The brain is susceptible to damage due to therapy The brain has a very limited capacity to repair itself Many drugs cannot cross the blood brain barrier to act on the tumor

Symptomatic therapyAnticonvulsantsCorticosteroids - reduce peritumoral edema

Glioblastoma multiformePalliative therapyto improve quality of life to achieve a longer survival time

Surgery1011 cells reduced to 109 cellsThe greater the extent of tumor removal, the longer the survival time

to take a section for a pathological diagnosis to remove some of the symptoms of a large mass pressing against the brain to remove disease before secondary resistance to radiotherapy and chemotherapy to prolong survival

Neuroblastoma

microscopic view of a typical neuroblastoma

with rosette formation

Neuroblastoma

most common extracranial solid cancer in childhood most common cancer in infancy50 % of neuroblastoma children < 2 years old

neuroendocrine tumor, arising from any neural crest element of the sympathetic nervous system

most frequently originates in one of the adrenal glands but can also develop in nerve tissues in the neck, chest, abdomen, or pelvis

Neuroblastoma

one of the few human malignancies known to demonstrate spontaneous regression from an undifferentiated state to a completely benign cellular appearance

three risk categories: - low - intermediate - high riskLow-risk disease is most common in infants highly curable with observation only or surgeryhigh-risk disease is difficult to cure even with the most intensive multi-modal therapies available

Etiology

not well understoodlinked to geneticsFamilial neuroblastoma very rare germline mutations in the anaplastic lymphoma kinase (ALK) gene

MILESTONESYear Name Findings

1864 German physician Rudolf Virchow

abdominal tumor in a child as a "glioma"

1891 German pathologist

Felix Marchand

characteristics of tumors from the sympathetic nervous system and the adrenal medulla

1901 William Pepper distinctive presentation of stage 4S in infants (liver but no bone metastases)

1910 James Homer Wright

the tumor to originate from primitive neural cells, named it neuroblastoma

circular clumps of cells in bone marrow samples which are now termed"Homer-Wright pseudorosettes"

ClassificationInternational Neuroblastoma Staging

SystemSTAGE TUMOUR

1 Localized tumor confined to the area of origin

2 A Unilateral tumor with incomplete gross resection; identifiable ipsilateral and contralateral lymph node negative for tumor

2 B Unilateral tumor with complete or incomplete gross resection; with ipsilateral lymph node positive for tumor; identifiable contralateral lymph node negative for tumor

3 Tumor infiltrating across midline with or without regional lymph node involvement; or unilateral tumor with contralateral lymph node involvement; or midline tumor with bilateral lymph node involvement

4 Dissemination of tumor to distant lymph nodes, bone marrow, bone, liver, or other organs except as defined by Stage 4S

4 S Age <1 year old with localized primary tumor as defined in Stage 1 or 2, with dissemination limited to liver, skin, or bone marrow (less than 10 percent of nucleated bone marrow cells are tumors)

International Neuroblastoma Risk Group Staging System (INRGSS)

STAGE GRADE

L 1 Localized disease without image-defined risk factors

L 2 Localized disease with image-defined risk factors

M Metastatic disease

M s Metastatic disease "special" where MS is equivalent to stage 4S

Signs and symptoms

Fatigue, loss of appetite, fever, and joint paindepend on primary tumor locations and metastases if present50 to 60% cases - present with metastasesA tumor in the abdomen, may cause swollen belly and constipation.A tumor in the chest may cause breathing problems.A tumors pressing on the spinal cord may cause weakness and thus an inability to stand, crawl, or walk.Bone lesions in the legs and hips may cause pain and limping.A tumor in the bones around the eyes or orbits may cause distinct bruising and swelling

Neuroblastomaoften spreads to other parts of the body before any symptoms are apparent50 - 60% - present with metastasesmost common location of primary tumor - adrenal glands 40% of localized tumors 60% of cases of widespread diseasecan also develop anywhere along the SNS chain

Site Percentage

neck 1%

chest 19%

abdomen 30% non-adrenal

pelvis 1%

no primary tumor

characteristic presentations Disease % of

casesPresentation

tumor spinal cord compression

5% transverse myelopathy

tumor vasoactive intestinal peptide secretion

4% treatment-resistant diarrhea

cervical tumor 2.4% Horner's syndrome

suspected paraneoplastic cause

1.3% opsoclonus myoclonus syndrome and ataxia

catecholamine secretion or renal artery compression

1.3% hypertension

Diagnosisusually confirmed by a surgical pathologistclinical presentation, microscopic findings, other laboratory tests

Serologyurine or bloodelevated levels of catecholamines or its metabolites - dopamine, - homovanillic acid (HVA), - vanillylmandelic acid (VMA)90% of cases of neuroblastoma

ImagingmIBG scan (meta-iodobenzylguanidine)mIBG-avid90 to 95% of all neuroblastomas

MechanismmIBG is taken up by sympathetic neurons functioning analog of neurotransmitter norepinephrineWhen it is radio-ionated with I-131 or I-123 (radioactive iodine isotopes) very good radiopharmaceutical for diagnosis and monitoring of response to treatment for this diseasehalf-life of I-123 - 13 hours, preferred isotope for imaging sensitivity and quality.half-life of I-131 - 8 days higher doses is an effective therapy as targeted radiation against relapsed and refractory neuroblastoma

Histology

microscopic view of stroma-rich ganglioneuroblastoma

tumor cells are typically described as small, round and blue, and rosette patterns (Homer-Wright pseudo-rosettes)

Neuroblastoma

one of the peripheral neuroblastic tumors (pNTs) wide pattern of differentiation benign ganglioneuroma stroma-rich ganglioneuroblastoma highly malignant neuroblastomaimportant prognostic factor with - age and - mitosis-karyorrhexis index (MKI)pathology classification systemInternational Neuroblastoma Pathology Committee (INPC, also called Shimada system) - Favorable - Unfavorable

ScreeningUrine catecholamine level can be elevated in pre-clinical neuroblastoma1980s - Japan, Canada, and Germany asymptomatic infants at three weeks, six months, 1 year

homovanillic acid and vanilmandelic acid 1984 - Japan six-month olds

Screening was halted 2004 - Canada and Germany - no reduction in deaths due to neuroblastoma, - increase in diagnoses that would have disappeared without treatment, unnecessary surgery and chemotherapy.

Treatmentlocalized lesion - generally curable

long-term survival for children with advanced disease > 18 months of age is poor despite aggressive multimodal therapy - intensive chemotherapy - surgery - radiation therapy - stem cell transplant - differentiation agent isotretinoin also called 13-cis-retinoic acid - immunotherapy with anti-GD2 monoclonal antibody therapy

Treatment options - different according to different risk categories low, intermediate, and high risk diseaseRisk assesment - age of the patient - extent of disease spread - microscopic appearance - genetic features - DNA ploidy and N-myc oncogene amplification (N-myc regulates microRNAs)

Risk % Treatment options Cure rates

low 37%

observed without any treatment at all or cured with surgery alone

90%

intermediate

18%

surgery and chemotherapy 70-90%

high 45%

intensive chemotherapy, surgery, radiation therapy, bone marrow / Hematopoietic stem cell transplantation biological-based therapy with 13-cis-retinoic acid (isotretinoin or Accutane) antibody therapy (cytokines GM-CSF and IL-2)

30%

Chemotherapy agents

platinum compounds cisplatin, carboplatin

alkylating agents cyclophosphamide, ifosfamide, melphalan

topoisomerase II inhibitor

etoposide

anthracycline antibiotics doxorubicin

vinca alkaloids vincristine

topoisomerase I inhibitors

topotecan and irinotecan (recurrent disease)

Medulloblastoma

highly malignant primary brain tumor originates in the cerebellum or posterior fossafamily of cranial primitive neuroectodermal tumors (PNET)Infratentorialinfratentorial PNETmost common PNET originating in the brain

All PNET tumors of the brain are invasive and rapidly growing tumors unlike most brain tumors spread through CSF metastasize to different locations in brain and spine

IncidenceBrain tumors second most common malignancy < 20 years of ageMedulloblastoma in children most common malignant brain tumor 14.5% in adults 2% of CNS malignancies

higher in males (62%) than females (38%)more prevalent in younger children Diagnostic age

% of medulloblastoma

Age Group

40% 0 - 5

31% 5 - 9

18.3% 10 - 14

12.7% 15 - 19

Pathogenesisusually form in the fourth ventricle, between the brainstem and the cerebellumexact cell of origin "medulloblast“ may be arised from cerebellar "stem cells" prevented from dividing and differentiating of normal cell types

highly characteristic of medulloblastoma perivascular pseudorosette and Homer-Wright rosette formation seen in up to half of the cases

Molecular genetics loss of genetic information on the distal part of chromosome 17, distal to the p53 geneneoplastic transformation of the undifferentiated cerebellar cells

may be associated with - Gorlin syndrome - Turcot syndrome - JC virus, the virus that causes multifocal leukoencephalopathy.

Clinical manifestation1 to 5 months before diagnosis increased intracranial pressure blockage of the fourth ventriclelistless,repeated episodes of vomitingmorning headachedevelop - stumbling gait - frequent falls - diplopia - papilledema - sixth cranial nerve palsy - positional dizziness - nystagmus - facial sensory loss or motor weakness Late - decerebrate attacks

Extraneural metastases to the rest of the body is rare - only after craniotomy

DiagnosisMRIdistinctive on T1 and T2-weighted MRI - with heterogeneous enhancement - typical location - adjacent to and - extension into the fourth ventricle

Histologicallysolidpink-gray in colorwell circumscribedvery cellularmany mitoseslittle cytoplasmhas tendency to form clusters and rosettes

Treatmentmaximal resection of the tumorto increase the disease-free survival - radiation to the entire neuraxis - chemotherapyIncreased ICP - corticosteroids or - ventriculoperitoneal shunt

Prognosiscombination therapy 5 year survival in > 80% of casesbetter prognosis presence of desmoplastic features - connective tissue formation poor prognosis - less than 3 years old - inadequate degree of resection - any CSF, spinal, supratentorial or systemic spread

Meningiomasecond most common primary tumor of the central nervous system arising from the arachnoid "cap" cells of the arachnoid villi in the meninges

usually - benign - can be malignant

Causesmost - sporadic some - familialrisk factor - radiation to the scalp genetic mutations - inactivation mutations in the neurofibromatosis 2 gene (merlin) on chromosome 22q

Signs and symptomsSmall tumors (e.g., < 2.0 cm) - usually incidental findings at autopsy - without having caused symptoms

Larger tumors - symptoms depending on the size and location

Increased ICP eventually occurs but is less frequent than in gliomas

Location being overlied Presentation

cerebrum Focal seizures

parasagittal frontoparietal region

Progressive spastic weakness in legs and incontinence

Sylvian tumors myriad motor, sensory, aphasic, and seizure symptoms depending on the location

Mechanismarise from arachnoidal cells most of which are near the vicinity of the venous sinuses (site of greatest prevalence for meningioma formation)

most frequently attached to the dura over the superior parasagittal surface of frontal and parietal lobes along the sphenoid ridge, in the olfactory grooves, the sylvian region, superior cerebellum along the falx cerebri, cerebellopontine angle, and the spinal cord

Tumour - gray, well-circumscribed, and space occupies dome-shaped, with the base lying on the dura

Cells - relatively uniform, (histologically) with a tendency to encircle one another - forming whorls and psammoma bodies (laminated calcific concretions) - have a tendency to calcify and are highly vascularized

Diagnosisvisualized with - contrast CT - MRI with gadolinium - arteriography lumbar puncture protein is usually elevated

A contrast enhanced CT scan of the braindemonstrating the appearance of a Meningioma

Classification of meningiomasbased upon the WHO classification system

majority of meningiomas are benign some - malignant

Type Grade %

Benign I 90% meningothelial, fibrous, transitional,

psammomatous, angioblastic

Atypical II 7% choroid, clear cell, atypical

Anaplastic/malignant

III 2% papillary, rhabdoid, anaplastic

Tumour type Mean overall survival

Mean relapse free survival

Atypical 11.9 years 11.5 years

Anaplastic/malignant 3.3 years 2.7 years

Treatment

Observation

Observation with close imaging follow-up can be used in select cases if a meningioma is small and asymptomatic

Observation is not recommended in tumors that are already causing symptoms

Conventional chemotherapy likely not effective

Tumour recurrence estimationSimpson Criteria

Simpson Grade

Completeness of Resection 10-year Recurrence

I complete removal including resection of underlying bone and associated

dura

9%

II complete removal + coagulation of dural attachment

19%

III complete removal w/o resection of dura or coagulation

29%

IV subtotal resection 40%

Radiation therapy

Gamma Knife radiosurgery small tumors located away from critical structures

fractionated external beam radiation

primary treatment for tumors that are surgically unresectable

inoperable for medical reasons

INDICATIONS FOR POST- OPERATIVE RADIATION

WHO GRADE TUMOUR RESECTIONS

Grade I subtotal (incomplete)

Grade II Complete or incomplete

Grade III Complete or incomplete

Secondaries brainLung- 40%Breast-19%[leptomeningeal deposits and

spinal cor compression are more]Melanoma-10%Gi tract-7%Others-17%