BRAIN TUMOURS

Brain tumor 
It is defined as any intracranial tumor caursed by abnormal and uncontrolled cell division, normally either - in the brain itself (neurons, glial cells (astrocytes, oligodendrocytes, ependymal cells), lymphatic tissue, blood vessels), - in the cranial nerves (myelin-producing Schwann cells), - in the brain envelopes (meninges), skull, pituitary and pineal gland, - or metastatic tumors

Brain tumor Primary (true) brain tumors are commonly located in the - posterior cranial fossa in children - anterior 2/3 of the cerebral hemispheres in adults, although they can affect any part of the brain.

AETIOLOGY PRIMARY .sporadic Environmentsl risk factor Smoking Diet Occupation Mobile phone No causative link proven Immunosupression Linked with Genetic abnormalities - .

Genetic Linking Disease Mutation Protein Tumour Neurofibromatosis Type 1 Neurofibromatosis Type 2 Li-Fraumeni¶s Syndrome Basal cell naevus syndrome Familial Adenomatous Polyposis Syndrome Chromosone 17 Nourofibromin Astrocytoma Neurofibroma Acoustic neuroma Meningioma Astrocytoma Chromosone 22 Schwannomin Chromosone 17 P 53 PTCH gene Medulloblastoma APC gene Medulloblastoma .

WHO CLASSIFICATION Glioma Astrocytomas Oligodendrogliomas Ependymoma Choroid Plexus Tumor Ganglioglioma Gangliocytoma Neuroblastoma Neuroepithelial Tumor Pineal Tumor Neuronal Tumor Medulloblastoma Nerve Sheath Tumor Meningeal Tumor Pituitary Tumor Germ Cell Tumor Lymphomas Vestibular Schwanoma Meningioma Germinoma Teratoma Craniopharyngioma Epidermoid Tumor Dermoid Tumor Colloid Cyst Tumor Like Malformation Metastatic Tumor Contiguous extension from regional Tumor ( Glomus Tumor ) .

GLIOMA  Arises from glial cells The most common site of gliomas is the brain Classification Classified . . . .by cell type.by location.by grade.

By cell type Glial Cells Astrocytes Oligodendrocytes Ependymal cells Different types of glia Glial Tumour Astrocytomas Oligodendrogliomas Ependymomas Mixed gliomas (oligoastrocytomas) .

By grade Grade Low-grade High-grade Differentiation well-differentiated (not anaplastic) undifferentiated (anaplastic) Type benign malignant Prognosis better worse HISTOLOGICAL CRITERIA Cellularity Nuclear pleomorphism Mitoses Vascular proliferation Necrosis .

By location Type Supratentorial Infratentorial Location above the tentorium below the tentorium Site in the cerebrum in the cerebellum Age mostly in adults (70%) mostly in children (70%) .

GLIOMA WHO CLASSIFICATION Astrocytomas Oligodendrogliomas Ependymoma Choroid Plexus Tumor GRADING GRADE I GRADE II GRADE III GRADE IV Pilocytic Astrocytoma Diffuse Astrocytoma Anaplastic Astrocytoma Glioblastoma Multiforme .

seizures. cranial nerve disorders visual loss pain. nausea. vomiting.Symptoms Depends on which part of the central nervous system is affected Affected organ brain glioma Symptoms increased intracranial pressure headaches. numbness in the extremities optic nerve glioma Spinal cord gliomas Drop metastases: not metastasize by the bloodstream but they can spread via the cerebrospinal fluid and cause "drop metastases" to the spinal cord . weakness.

Pathology High-grade gliomas highly-vascular tumors tendency to infiltrate extensive areas of necrosis and hypoxia breakdown of the blood-brain barrier almost always recur even after complete surgical excision Low-grade gliomas grow slowly over many years followed without treatment unless they grow and cause symptoms .

Surgery .grade of malignancy combined approach .Radiation therapy external beam radiation stereotactic approach using radiosurgery Spinal cord tumors .surgery + radiation .Treatment depends on .location.Chemotherapy Temozolomide able to cross the blood-brain barrier . .cell type .

1 year after diagnosis 25% . 2009 at his home in Hyannis Port.Prognosis Gliomas cannot be cured High-grade gliomas Poor prognosis 50% . Massachusetts .2 years after diagnosis anaplastic astrocytoma survive about three years Glioblastoma multiforme has a worse prognosis PROGNOSIS Histological grade Cell type Tumor size Patient s factor Senator Kennedy passed away from the malignant glioma tumor on August 25.

survival 3 .< 6 % of Intracranial tumors .ASTROCYTOMA Astrocytomas originate in star-shaped brain cells called astrocytes most common primary CNS malignancy 75% of neuroepithelial tumors low grade grade (IV) .12 months .20 % of Intracranial tumors .

ASTROCYTOMA Classification I I A II III IV V VI VI A VI B VII Tumour Pilocytic astrocytoma Pilomyxoid astrocytoma Subependymal giant cell astrocytoma Pleomorphic xantho astrocytoma Diffuse astrocytoma Anaplastic astrocytoma Glioblastoma Giant cell glioblastoma Gliosarcoma Gliomatosis cerebri Grade I II I II II III IV IV IV III Low Low Low Low Low High High High High High .

20% of all intracranial tumors Classified .most common .Glioblastoma .Gliosarcoma .Giant cell glioblastoma .most aggressive .Glioblastoma multiforme standard name .52% of all parenchymal brain tumor .

diet. Von Hippel-Lindau disease. Latinos. .low-grade astrocytoma (brain tumor) . cellular phones. without any genetic predisposition No links . Turcot syndrome . Asians Linked .polyvinyl chloride . Li-Fraumeni syndrome.Glioblastoma multiforme Causes Sporadic .ionizing radiation .smoking. Tuberous sclerosis. electromagnetic fields Sex: male Age: over 50 years old Ethnicity: Caucasians.genetic disorder Neurofibromatosis.cytomegalovirus.

and hemiparesis progressive memory.Glioblastoma multiforme Symptoms depends highly on the location asymptomatic condition seizure. personality. headache. or neurological deficit due to temporal and frontal lobe involvement Diagnosis MRI ring-enhancing lesions Definitive diagnosis of a suspected GBM on CT or MRI stereotactic biopsy or craniotomy with tumor resection . nausea and vomiting.

Glioblastoma multiforme Sagittal MRI with contrast glioblastoma WHO grade IV in a 15-year-old boy .

grow quickly extend 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 bilateral classic infiltration across the corpus callosum butterfly (bilateral) glioma Mass effect .Glioblastoma multiforme Pathogenesis presence of small areas of necrotizing tissue that is surrounded by anaplastic cells (pseudopalisading necrosis) differentiates Grade 3 astrocytomas presence of hyperplastic blood vessels secondary GBM degeneration of lower grade gliomas more common in younger patients In the cerebral white matter.tumor and edema compress the ventricles and cause hydrocephalus .

Glioblastoma multiforme Treatment very difficult The 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 therapy Anticonvulsants Corticosteroids .reduce peritumoral edema .

Glioblastoma multiforme Palliative therapy to improve quality of life to achieve a longer survival time Surgery 1011 cells reduced to 109 cells The 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 .

Glioblastoma multiforme Radiotherapy radiotherapy after surgery reduce the tumor size to 107 cells A total radiation dose of 60±65 Gy .optimal Chemotherapy standard of care for glioblastoma is chemotherapy during and after radiotherapy chemotherapy after surgery and radiotherapy reduce the tumor size to 106 cells Temozolomide during radiotherapy for six months post radiotherapy .

Glioblastoma multiforme Prognosis Median survival time from the time of diagnosis without any treatment .3 months glioblastoma multiforme prognosis depends on .Karnofsky Performance Score (KPS) .age of the patient .treatment .

derives from the Greek roots 'oligo' meaning few 'dendro' meaning trees They occur primarily in adults (9.viral cause .irradiation of pituitary adenoma .4% of all primary brain ) in children (4% of all primary brain tumors) The average age at diagnosis is 35 years etiology of oligodendrogliomas is unknown linked .Oligodendroglioma a type of glioma that are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell.

affecting personality Headaches combined with increased intracranial pressure Depending on the location of the tumor. . relies on histopathologic examination (biopsy examination). location.seizure frontal lobe .Symptoms first symptom . heter/homogeneity) final diagnosis of this tumor. any neurological deficit (CT) or (MRI) scan is necessary to characterize the anatomy of this tumor (size.

Microscopic Appearance characteristic fried egg-like cells. small. with clear cytoplasm and well-defined cell borders. compact nuclei and a small amount of eosinophilic cytoplasm Micrograph of an oligodendroglioma showing the characteristic branching. with clear cytoplasm and well-defined cell borders. H&E stain. . H&E stain. composed of cells with small to slightly enlarged round nuclei with dark. chicken wire-like blood vessels and fried egg-like cells.

5 years for grade III very high (almost uniform) rate of recurrence gradually increase in grade over time .Oligodendroglioma Tumour Oligodendroglioma Anaplastic oligodendroglioma Grading II III Incurable slowly growing with prolonged survival median survival times -11.6 years for grade II -3.

Temozolomide standard dosing schedule 5 consecutive days of daily dosing during 28 day cycles Surgery Because of their diffusely infiltrating nature.TREATMENT Chemotherapy . oligodendrogliomas cannot be completely resected not curable by surgical excision Surgery may be followed up by chemotherapy. radiation Stereotactic surgery small tumors that have been diagnosed early .

pelvic cavity Syringomyelia can be caused by an ependymona Ependymomas are also seen with Neurofibromatosis Type II 5% of adult intracranial gliomas 10% of childhood tumors of (CNS) peak at age 5 years and then again at age 35 About 85% of ependymomas benign myxopapillary ependymoma (MPE) usually arise from the floor of the fourth ventricle .intracranial in adults . a tissue of the central nervous system location in children .fourth ventricle can occur in .spinal common intracrania location .Ependymoma a tumor that arises from the ependyma.

Symptoms by obstructing the flow of cerebrospinal fluid headache. seeing vertical or horizontal lines when in bright light. uncontrollable twitching. temporary memory loss . temporary inability to distinguish colors. nausea and vomiting hydrocephalus Other symptoms loss of appetite. difficulty sleeping.

Ependymoma TUMOUR Subependymoma Myxopapillary ependymoma Ependymoma Anaplastic ependymoma GRADE I I II III .

may spread through the cerebrospinal fluid usually require radiation therapy .TREATMENT well-differentiated ependymomas radiation therapy only other ependymomas total surgical removal is the preferred treatment cannot be totally removed .treated similarly to medulloblastoma combination of radiation therapy and chemotherapy in infants and children younger than 5 years of age.require radiation therapy malignant (anaplastic) varieties of this tumor malignant ependymoma Ependymoblastoma .

cause obstruction well-encapsulated tumor generally has a very benign course .Subependymoma a variant of the ependymoma arise in the fourth ventricle in the septum pellucidum cervical spinal cord over 40 years of age men > women subependymal giant-cell astrocytoma. (giant-cell glioma) is typically associated with tuberous sclerosis but can occur independent of that condition Arising in the walls of the lateral ventricles over the basal ganglia .

may be an unusual form of teratoma or may be confused with a sacrococcygeal teratoma .Extraspinal ependymoma (EEP) also known as extradural ependymoma.

Choroid plexus Tumours Tumour Grade Choroid plexus papilloma I Atypical choroid plexus papilloma II Choroid plexus carcinoma III .

slow-growing.Choroid plexus papilloma rare. histologically benign intracranial tumor commonly located in the ventricular system of the choroid plexus obstruct the cerebrospinal fluid flow causing increased intracranial pressure .

4-0.6% of all intracranial neoplasms most commonly affects young children under the age of 5 mean patient age of 5.Pathophysiology neuroectodermal in origin similar in structure to a normal choroid plexus may be created by epithelial cells of choroid plexus Frequency and age affected 0.2 years .

91% common symptoms vomiting. homonymous visual field defects and headache Surgical treatment benign tumors usually cured by surgery malignant progression has been rarely reported .Signs and symptoms Signs of increased intracranial pressure .

areas of neutropil + tumor cells with small round nuclei .well-differentiated .spread through CSF .necrosis and mitoses .benign.high grade tumor . slowly-growing tumor .densely packed small cells .no mitoses or necrosis Pineoblastoma .may occur inRetinoblastoma patients .also known as a pinealocytoma .more common in children .Pineal tumours arise from specialized cells of pineal gland -Pineocytes Pineocytoma .low grade tumor .

Pineal tumours TUMOUR Pineocytoma Pineal parenchymal tumour of intermediate differentiation Pineoblastoma Papillary tumour of the pineal region GRADE I II III IV II III .

between the two hemispheres. . tucked in a groove where the two rounded thalamic bodies join midline structure.Pineal gland reddish-gray size of a pea (8 mm) shaped like a tiny pine cone pineal body.between the laterally positioned thalamic bodies. (often calcified ) produces melatonin. epiphysis cerebri. epiphysis or the "third eye located .just retro-dorsal to the superior colliculus . part of the epithalamus. often seen in plain skull X-rays. located near to the center of the brain. a hormone that affects the modulation of wake/sleep patterns and photoperiodic (seasonal) functions .behind and beneath the stria medullaris.

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or in the spinal cord often associated with seizures second most common cause of spinal cord tumors in children mature ganglion cells clustered with neoplastic glial cells .Ganglioglioma tumour that arises from ganglion cells in CNS temporal lobe can occur anywhere in the brain.

Dysplastic Cerebellar Ganglioglioma (Lhermitte-Duclos disease) .

Neuronal and mixed neuronal-glial tumours Tumour Gangliocytoma Ganglioglioma Anaplastic ganglioglioma Desmoplastic infantile astrocytoma and ganglioglioma Dysembryoplastic neuroepithelial tumour Central neurocytoma Extraventricular neurocytoma Cerebellar liponeurocytoma Paraganglioma of the spinal cord Papillary glioneuronal tumour Rosette-forming glioneuronal tumour of the fourth ventricle Grade I I II I I II II II I I I .

Neuroblastoma microscopic view of a typical neuroblastoma with rosette formation .

chest. or pelvis . 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.Neuroblastoma most common extracranial solid cancer in childhood most common cancer in infancy 50 % of neuroblastoma children < 2 years old neuroendocrine tumor. abdomen.

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

Etiology not well understood linked to genetics Familial neuroblastoma very rare germline mutations in the anaplastic lymphoma kinase (ALK) gene .

first year of life some cases are congenital  10%  < 2% > 5 years of age > 18 years of age .Epidemiology 6-10% 15% of all childhood cancers of cancer deaths in children annual mortality rate 10 per million in 4 per million in highest incidence 0 ± 4 year old age group 4 ± 9 year old age group .

named it neuroblastoma circular clumps of cells in bone marrow samples which are now termed "Homer-Wright pseudorosettes" 1901 1910 James Homer Wright .MILESTONES Year 1864 Name German physician Rudolf Virchow German pathologist Felix Marchand William Pepper Findings abdominal tumor in a child as a "glioma" 1891 characteristics of tumors from the sympathetic nervous system and the adrenal medulla distinctive presentation of stage 4S in infants (liver but no bone metastases) the tumor to originate from primitive neural cells.

German physician Rudolf Virchow the first to describe an abdominal tumor in a child as a "glioma" .

or bone marrow (less than 10 percent of nucleated bone marrow cells are tumors) 2B 3 4 4S . or other organs except as defined by Stage 4S Age <1 year old with localized primary tumor as defined in Stage 1 or 2. identifiable contralateral lymph node negative for tumor Tumor infiltrating across midline with or without regional lymph node involvement. bone marrow.Classification International Neuroblastoma Staging System STAGE 1 2A TUMOUR Localized tumor confined to the area of origin Unilateral tumor with incomplete gross resection. bone. or unilateral tumor with contralateral lymph node involvement. identifiable ipsilateral and contralateral lymph node negative for tumor Unilateral tumor with complete or incomplete gross resection. or midline tumor with bilateral lymph node involvement Dissemination of tumor to distant lymph nodes. with dissemination limited to liver. with ipsilateral lymph node positive for tumor. liver. skin.

International Neuroblastoma Risk Group Staging System (INRGSS) STAGE L1 L2 M Ms GRADE Localized disease without image-defined risk factors Localized disease with image-defined risk factors Metastatic disease Metastatic disease "special" where MS is equivalent to stage 4S .

and joint pain depend on primary tumor locations and metastases if present 50 to 60% cases .present with metastases A tumor in the abdomen. A tumor in the chest may cause breathing problems.Signs and symptoms Fatigue. A tumors pressing on the spinal cord may cause weakness and thus an inability to stand. Bone lesions in the legs and hips may cause pain and limping. loss of appetite. crawl. A tumor in the bones around the eyes or orbits may cause distinct bruising and swelling . may cause swollen belly and constipation. fever. or walk.

60% .Neuroblastoma often spreads to other parts of the body before any symptoms are apparent 50 .adrenal glands 40% of localized tumors 60% of cases of widespread disease can also develop anywhere along the SNS chain Site neck chest abdomen pelvis no primary tumor Percentage 1% 19% 30% non-adrenal 1% .present with metastases most common location of primary tumor .

4% Horner's syndrome suspected paraneoplastic cause 1.characteristic presentations Disease % of cases 5% Presentation tumor spinal cord compression transverse myelopathy tumor vasoactive intestinal peptide secretion cervical tumor 4% treatment-resistant diarrhea 2.3% .3% opsoclonus myoclonus syndrome and ataxia hypertension catecholamine secretion or renal artery compression 1.

microscopic findings. other laboratory tests Serology urine or blood elevated levels of catecholamines or its metabolites .homovanillic acid (HVA).vanillylmandelic acid (VMA) 90% of cases of neuroblastoma .Diagnosis usually confirmed by a surgical pathologist clinical presentation. .dopamine. .

13 hours. preferred isotope for imaging sensitivity and quality. half-life of I-131 .Imaging mIBG scan (meta-iodobenzylguanidine) mIBG-avid 90 to 95% of all neuroblastomas Mechanism mIBG is taken up by sympathetic neurons functioning analog of neurotransmitter norepinephrine When 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 disease half-life of I-123 .8 days higher doses is an effective therapy as targeted radiation against relapsed and refractory neuroblastoma .

and rosette patterns (Homer-Wright pseudo-rosettes) . round and blue.Histology microscopic view of stroma-rich ganglioneuroblastoma tumor cells are typically described as small.

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

and Germany asymptomatic infants at three weeks. 1 year homovanillic acid and vanilmandelic acid 1984 . .increase in diagnoses that would have disappeared without treatment.no reduction in deaths due to neuroblastoma. .Screening Urine catecholamine level can be elevated in pre-clinical neuroblastoma 1980s . Canada. six months.Canada and Germany . unnecessary surgery and chemotherapy.Japan.Japan six-month olds Screening was halted 2004 .

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

genetic features . 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) 90% 70-90% 30% .microscopic appearance .different according to different risk categories low. and high risk disease Risk assesment . surgery.age of the patient .extent of disease spread .DNA ploidy and N-myc oncogene amplification (N-myc regulates microRNAs) Risk low intermediate high % Treatment options Cure rates 37% observed without any treatment at all or cured with surgery alone 18% surgery and chemotherapy 45% intensive chemotherapy. intermediate. radiation therapy.Treatment options .

carboplatin cyclophosphamide.Chemotherapy agents platinum compounds alkylating agents topoisomerase II inhibitor anthracycline antibiotics vinca alkaloids topoisomerase I inhibitors cisplatin. melphalan etoposide doxorubicin vincristine topotecan and irinotecan (recurrent disease) . ifosfamide.

Medulloblastoma highly malignant primary brain tumor originates in the cerebellum or posterior fossa family of cranial primitive neuroectodermal tumors (PNET) Infratentorial infratentorial PNET most 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 .

3% 12.7% Age Group 0-5 5-9 10 .14 15 .5% in adults 2% of CNS malignancies higher in males (62%) than females (38%) more prevalent in younger children Diagnostic age % of medulloblastoma 40% 31% 18.Incidence Brain tumors second most common malignancy < 20 years of age Medulloblastoma in children most common malignant brain tumor 14.19 .

the virus that causes multifocal leukoencephalopathy.Gorlin syndrome .Pathogenesis usually form in the fourth ventricle. distal to the p53 gene neoplastic transformation of the undifferentiated cerebellar cells may be associated with . .JC virus. between the brainstem and the cerebellum exact cell of origin "medulloblast³ 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.Turcot syndrome .

repeated episodes of vomiting morning headache develop .frequent falls .stumbling gait .decerebrate attacks Extraneural metastases to the rest of the body is rare .positional dizziness .sixth cranial nerve palsy .nystagmus .diplopia .papilledema .facial sensory loss or motor weakness Late .only after craniotomy .Clinical manifestation 1 to 5 months before diagnosis increased intracranial pressure blockage of the fourth ventricle listless.

typical location .with heterogeneous enhancement .adjacent to and .Diagnosis MRI distinctive on T1 and T2-weighted MRI .extension into the fourth ventricle Histologically solid pink-gray in color well circumscribed very cellular many mitoses little cytoplasm has tendency to form clusters and rosettes .

chemotherapy Increased ICP .radiation to the entire neuraxis .Treatment maximal resection of the tumor to increase the disease-free survival .corticosteroids or .inadequate degree of resection . supratentorial or systemic spread .connective tissue formation poor prognosis . spinal.any CSF.ventriculoperitoneal shunt Prognosis combination therapy 5 year survival in > 80% of cases better prognosis presence of desmoplastic features .less than 3 years old .

Embryonal tumours Tomour Medulloblastoma CNS primitive neuroectodermal tumour (PNET) Atypical teratoid / rhabdoid tumour Grade IV IV IV .

Schwannoma Neurilemmoma benign nerve sheath tumor composed of Schwann cells which normally produce the insulating myelin sheath covering peripheral nerves very homogeneous tumors consisting only of Schwann cells tumor cells always stay on the outside of the nerve but the tumor itself may either push the nerve against a bony structure thereby possibly causing damage relatively slow growing mostly benign < 1% become malignant degenerating into a form of cancer known as neurofibrosarcoma can arise from a genetic disorder called neurofibromatosis can be removed surgically but can then recur .

III . IV . III II . II .Tumours of the cranial and paraspinal nerves Tumour Schwannoma Neurofibroma Perineurioma Malignant peripheral nerve sheath tumour (MPNST) Grade I I I .

Meningeal tumours WHO classification Tumour Meningioma Atypical meningioma Anaplastic / malignant meningioma Haemangiopericytoma Anaplastic haemangiopericytoma Haemangioblastoma Grade I II III II III I .

can be malignant Causes most .sporadic some .Meningioma second most common primary tumor of the central nervous system arising from the arachnoid "cap" cells of the arachnoid villi in the meninges usually .familial risk factor genetic mutations .benign .radiation to the scalp .inactivation mutations in the neurofibromatosis 2 gene (merlin) on chromosome 22q .

sensory. < 2.Signs and symptoms Small tumors (e.usually incidental findings at autopsy .0 cm) .without having caused symptoms Larger tumors . and seizure symptoms depending on the location Presentation .g.symptoms depending on the size and location Increased ICP eventually occurs but is less frequent than in gliomas Location being overlied cerebrum Focal seizures parasagittal frontoparietal region Progressive spastic weakness in legs and incontinence Sylvian tumors myriad motor. aphasic..

superior cerebellum along the falx cerebri. and the spinal cord Tumour . in the olfactory grooves.relatively uniform.Mechanism arise 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. with the base lying on the dura Cells . cerebellopontine angle. well-circumscribed.have a tendency to calcify and are highly vascularized . (histologically) with a tendency to encircle one another . and space occupies dome-shaped.forming whorls and psammoma bodies (laminated calcific concretions) .gray. the sylvian region.

contrast CT .Diagnosis visualized with .MRI with gadolinium .arteriography  lumbar puncture protein is usually elevated A contrast enhanced CT scan of the brain demonstrating the appearance of a Meningioma .

transitional.malignant Type Benign Grade I % 90% meningothelial. angioblastic choroid. anaplastic Atypical Anaplastic/malignant II III 7% 2% Tumour type Mean overall survival Mean relapse free survival Atypical Anaplastic/malignant 11.5 years 2.Classification of meningiomas based upon the WHO classification system majority of meningiomas are benign some . atypical papillary.7 years .9 years 3. fibrous. psammomatous. clear cell.3 years 11. rhabdoid.

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

Surgical resection
can usually be surgically resected with permanent cure if the tumor is - superficial on the dural surface - and easily accessible Transarterial embolization standard preoperative procedure in the preoperative management If invasion of the adjacent bone occurs total removal is nearly impossible Malignant transformation is rare

probability of tumor recurrence estimated - by the tumor's WHO Grade and - by the extent of surgery by the Simpson Criteria

Tumour recurrence estimation Simpson Criteria
Simpson Grade Completeness of Resection 10-year Recurrence 9%

I

complete removal including resection of underlying bone and associated dura complete removal + coagulation of dural attachment complete removal w/o resection of dura or coagulation subtotal resection

II

19%

III

29%

IV

40%

Radiation therapy INDICATIONS FOR POST.OPERATIVE RADIATION WHO GRADE Grade I Grade II Grade III TUMOUR RESECTIONS subtotal (incomplete) Complete or incomplete Complete or incomplete Gamma Knife radiosurgery small tumors located away from critical structures primary treatment for tumors that are surgically unresectable inoperable for medical reasons fractionated external beam radiation .

aneurysm .benign Metastasis .craniopharyngioma .Rathke¶s cleft cyst .meniongioma .PITUITARY TUMOUR 10 ± 50 % of all cranial tumour Majority .may occur especially in elderly One type of sellar region tumour Differential diagnosis of sellar region mass .

2¶) ACTH producing tumour Cushing syndrome GH secreting tumour Acromegaly gigantism Mass effect . VI disorder Endocrine disturbance TUMOUR PRESENTATIONS Prolactinoma Galactorrhoea Amenorrhoea (P¶.pressure on optic chiasma . IV.cranial nerve III.Clinical features .bitemporal hemianopia .

impotence Pathology Cushing's disease Acromegaly (gigantism) hyperthyroidism % 15 % 20 % 1% 1.Clinical syndrome for secretory pituitary tumor Type of adenoma corticotrophic somatotrophic thyrotrophi gonadotrophic lactrotrophic / prolactinomas null cell Secretion (ACTH) (POMC) (GH) (TSH) (LH) (FSH) prolactin subunit Staining basophilic acidophilic basophilic basophilic acidophilic synaptophysin galactorrhea. hypogonadism.2 % 40 % 20 % . amenorrhea.infertility.

Tumours of the sellar region Tumour Craniopharyngioma Granular cell tumour of the neurohypophysis Pituicytoma Spindle cell oncocytoma of the adenohypophysis Grade I I I I .

opthalmoplegia .sudden onset of .altered concious level Sudden onset of headache & meningism similar to SAH Management .urgent decompression .preoperative resuscitation .headache .visual loss .Pituitary apoplexy Hemorrhagic infarction of pituitary tumour Presentations .

MRI scanof pituitary region Baseline assesment of pituitary function .prolactin .Growth hormopne .Leutinizing hormone .Insulin like growth factor 1 .Folicular stimulating hormone .Investigation Formal visual field & acuity test Imaging .Thyroid function .fasting serum .urinary cortisol .

endocrine status .Diagnosis of ACTH secreting tumour is difficult .Cortisol deficiency must be corrected (preop) .Investigation Essential assesment .high Prolactin level .Prolactinoma .ACTH cortisol axis .Petrosal sinus sampling .Dexamethasone supression test .Prolactin .preclude the need for surgery (Pituitary stalk compression moderately elevate prolactin level) .

Endocrinologist .Neurosurgeon .Cabergoline .Octreotide .Management AIM Close to alleviate mass effect restore or replace endocrine function prevent recurrence co-operation of .Dopamine agonist .initial treatment .Somatostatin .medically analogues .Dopamine agonist Prolactinoma .Bromocryptine .medically Growth hormone secreting tumour .

Surgical treatment Transsphenoidal Surgery .operating microscope .Diabetes insipidus Determination of surgical success (transient) Normalisation of endocrine dysfunction Lack & Recurrence Vs condition being treated Size of tumor .pituitary stalk manipulation .endoscope assisted Large tumour with suprssellar extension .Craniotomy Transsphenoidal Surgery Complication Complication CSF leakage Visual deterioration Major vessel injury Pan hypo pituitarism Percentage 3 % 1 % 1 % 1 % .

and a number of others 15 % Some types of cancers only spread to the brain infrequently . testicular and germ cell tumors.prostate cancer unknown primary (CUP) origin Infrequently. certain sarcomas. yet the original site or location of the tumor is unknown 10 . a tumor can spread to the brain.30% of adult cancers .colon cancer. 10 30 % melanoma.Metastatic brain tumor A metastatic brain tumor is brain cancer that has spread from another part of the body Causes Many tumor or cancer types can spread to the brain most common lung cancer. bladder cancer. 40 % breast cancer. very rarely . 5 15 % kidney cancer.

have not been previously diagnosed Lung.80 % of metastatic brain tumors in women incidence increased over time .INCIDENCE more common than primary brain tumors about one-fourth of all cancers that metastasize 10 .approximately one month without any treatment two months with corticosteroids three to six months with cranial irradiation .improvements in treatment of primary tumour and systemic disease median survival . lung.advances in neuroimaging procedures .30% of adult cancers 81 % . colon and renal cancers .80 % of metastatic brain tumors in men Breast. colon and melanoma cancers .diagnosed after their primary cancer has been diagnosed and treated 35 % .

cerebellum (13-16%) .brain stem (3%) usually solid and spherical in shape with well-defined margins. center is often soft and filled with dead cells zone of active tumor cells ( ringlike structure on the scan ) commonly grow in the junction of white and grey matter (most blood vessels) 50% of the time multiple tumors usually accompanied by widespread edema exact diagnosis .cerebrum (80%). .CHARACTERISTICS OF METASTATIC BRAIN TUMORS in the .biopsy sometimes recommended to eliminate the chance of misdiagnosis .

neurologic changes specific to the location of the tumor . MRI . .specimen from the tumor during surgery or CT scan-guided biopsy is used to confirm the exact type of tumor If the primary tumor can be located outside of the brain. chest x-ray. Histology . rapid decline in neurologic function Investigation CT scan or MRI of the brain confirm the diagnosis identify the location of the tumor. EEG may reveal abnormalities.Signs of increased ICP .Exams and Tests examination . abdomen. and other tests are performed to look for the original site of the tumor. CT scans of the chest.to test the cerebral spinal fluid. and pelvis.Some -no symptoms until they are very large. mammogram. primary tumor is usually biopsied rather than the brain tumor.better Cerebral angiography show a space-occupying mass which may or may not be highly vascular (filled with blood vessels). lumbar puncture . Then.

initial site of the tumor .relief of symptoms .total dose of 3000 cGy to the entire brain .followed by a booster dose of 900 cGy to the tumor . for two weeks .often follows brain surgery standard treatment .whole brain is often used .Treatment .comfort Radiation .especially > one tumor depends on external irradiation .size and type of the tumor .whole brain radiotherapy (WBRT) .300 cGy portions five days a week.general health of the person goals of treatment .improved functioning .

surgery could be considered. 5-FU.. Tamoxifen .metastatic from breast cancer cyclophosphamide.reduce pressure and relieve symptoms in cases when the tumor cannot be removed Karnofsky Performance Scale 100 Normal. if other factors are favorable Surgery Chemotherapy .some .does not pass the blood brain barrier . minor signs or symptoms of disease 80 Normal activity with effort.not as helpful as surgery or radiation for many types of cancer.completely removed . some signs or symptoms of disease 70 Cares for self. unable to carry on normal activity or to do active work 60 Requires occasional assistance but is able to care for most of needs > 60 or 70.a single lesion and when there is no cancer elsewhere in the body .debulked (reduce tumor size) .deep or that infiltrate brain tissue . and methotrexate. . no evidence of disease 90 Able to carry on normal activity. no complaints.

relief of pain and other symptoms Comfort measures.Medications for some symptoms of a brain tumor Corticosteroids such as dexamethasone to reduce brain swelling Osmotic diuretics such as urea or mannitol to reduce brain swelling Anticonvulsants such as phenytoin to reduce seizures Pain medication Antacids or antihistamines to control stress ulcers multiple metastases (widespread cancer) treatment . safety measures. such as power of attorney. in cases where continued physical or intellectual decline is likely . physical therapy. occupational therapy -to improve the patient's quality of life. Legal advice may be helpful in forming advanced directives.

2:1 .Brain tumors in infants and children Most common solid tumor in children second most common tumour (haematological dis-orders) 20 per cent of childhood malignancies 2 5 cases per 100 000 male preponderance of 1.

primitive neuroectodermal tumor .neuroectodermal origin .teratoma .high grade astrocytoma .Pilocystic astrocytoma Germ cell tumors.mostly in supratentorial tumor types .choroid plexus papilloma / carcinoma Children < 2 years . including teratoma 3% of pediatric primary brain tumors about 70% of brain tumors .Ependymoma .mostly in infratentirial tumor types .Brain tumors in infants and children Neonates .medulloblastoma .

varies with .type of cancer .Brain tumors in infants and children survival rate in children . without contrast .approximately 60% .age of onset: younger patients have higher mortality brain-stem glioma in four year old MRI sagittal.

disease process is focal rather than diffuse Focal disease processes include tumors or infarctions.Focal neurologic signs perceptual or behavioral impairments which are caused by lesions in a particular area of the central nervous system. Diffuse disease processes include meningitis or encephalitis .

described as an expressive aphasia (Broca's aphasia) focal seizures which can spread to adjacent areas (Jacksonian seizure) grand mal or tonic-clonic seizure . and may include many special types of deficit.Frontal lobe signs usually involve the motor system. resistance to passive movements of the limbs (hypertonia) paralysis of a limb (monoparesis) or a larger area on one side of the body (hemiparesis) paralysis head and eye movements inability to express oneself linguistically. depending on which part of the frontal lobe is affected unsteadiness in walking muscular rigidity.

reappearance of primitive reflexes snout reflex. loss of initiative and concern. general retardation "frontal release" signs.Frontal lobe signs changes in personality disinhibition. apathy. rage without provocation. akinetic mutism. the grasp reflex. inappropriate jocularity.e. i. palmar-mental reflex unilateral loss of smell (anosmia) .

Parietal lobe signs usually involve somatic sensation impairment of tactile sensation impairment of proprioception. loss of ability to read.e. dysgraphia.e. write or calculate (dyslexia. postural sensation and sensation of passive movement sensory and visual neglect syndromes. i. i. dyscalculia) loss of ability to find a defined place (geographical agnosia) loss of ability to identify objects based on touch (astereognosia) . inability to pay attention to things in certain parts of the person's sensory or spatial environment. This can be as extreme as denial of a limb.

described as a sensory aphasia (Wernicke's aphasia) amnesia.Temporal lobe signs usually involve auditory sensation and memory deafness without damage to the structures of the ear. multimodal hallucinations complex partial seizures (temporal lobe epilepsy) .or short-term memory or both) other memory disturbances such as deja vu complex. described as cortical deafness tinnitus. memory loss (affecting either long. auditory hallucinations loss of ability to comprehend music or language.

such as zig-zags and flashes. (In contrast. displaying elementary forms. temporal lobe visual hallucinations display complex forms. and fill the entire visual field.Occipital lobe signs usually involve visual sensation total loss of vision (cortical blindness) loss of vision with denial of the loss (Anton's syndrome) loss of vision on one side of the visual field of both eyes (homonymous hemianopsia) visual agnosias.e. colors. or faces visual illusions such as micropsia (objects appear smaller) and macropsia (objects appear larger) visual hallucinations.) . inability to recognize familiar objects. i. in one half of the visual field only for each eye.

e.Cerebellar signs involve balance and coordination unsteady and clumsy motion of the limbs or torso (ataxia) inability to coordinate fine motor activities (intention tremor) e.g. inability to rapidly flip the hands involuntary left-right eye movements (nystagmus) .g. "past-pointing" (pointing beyond the finger in the finger-nose test) inability to perform rapid alternating movements (dysdiadochokinesis).

Brainstem signs
specific sensory and motor abnormalities depending on which fiber tracts and cranial nerve nuclei are affected

Spinal cord signs
generally involve unilateral paralysis with contralateral loss of pain sensation

Stereotactic surgery 

The first stereotactic frame is attributed to Horsley and Clark principle of stereotaxy is that described by Descartes; any point in space can be identified in relation to three planes running perpendicular to each other

frame-based stereotaxy
a device is fixed to the head, so that the co-ordinates of any point within the confines of the frame itself or an imaginary extension of it can be accurately localised. This requires CT or MRI to be per-formed after the frame has been applied to the head, so that the position of the region of interest can be related to the frame 
used not only for tumour biopsy but also for localising cranio-tomies for minimally invasive excision of tumours for catheter placement, for drug or isotope delivery or cyst drainage

frameless stereotaxy An extension of the stereotactic process is to obtain a preoperative scan without the use of a frame and then use a mathematical algorithm to correlate with the surface markings of the skull at the time of surgery less clumsy and time consuming .

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