a case of cns tumour
TRANSCRIPT
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DR.G .BALAJIPROF.DR.G.SUNDARAMURTHY’S unit
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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.
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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
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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.
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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
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Provisional diagnosis SEIZURE DISORDER- LATE ONSET ? CAUSE
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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
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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
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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.
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NEUOSURGEON’S OPINION:ANAPLSTIC ASTROCYTOMA/ GLIOMA-low
gradePt was advised surgeryPt was not willing for surgery at Stanley .
Discharged at request.
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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.
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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
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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
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T
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CNS TUMOURS
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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 )
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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
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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
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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.
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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
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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
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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%)
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By cell type
Glial Cells Glial Tumour
Astrocytes Astrocytomas
Oligodendrocytes Oligodendrogliomas
Ependymal cells Ependymomas
Different types of glia Mixed gliomas (oligoastrocytomas)
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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
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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
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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
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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.
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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
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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.
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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
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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
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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
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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.
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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).
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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.
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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
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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
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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
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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
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Glioblastoma multiforme
standard name - “Glioblastoma”- most common - most aggressive - 52% of all parenchymal brain tumor- 20% of all intracranial tumorsClassified - Giant cell glioblastoma - Gliosarcoma
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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
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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
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Glioblastoma multiforme
Sagittal MRI with contrast glioblastoma
WHO grade IV in a 15-year-old boy
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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
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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
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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
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Neuroblastoma
microscopic view of a typical neuroblastoma
with rosette formation
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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
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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
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Etiology
not well understoodlinked to geneticsFamilial neuroblastoma very rare germline mutations in the anaplastic lymphoma kinase (ALK) gene
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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"
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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)
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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
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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
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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
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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
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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
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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
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Histology
microscopic view of stroma-rich ganglioneuroblastoma
tumor cells are typically described as small, round and blue, and rosette patterns (Homer-Wright pseudo-rosettes)
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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
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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.
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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
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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%
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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)
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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
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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
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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.
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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%
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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
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Secondaries brainLung- 40%Breast-19%[leptomeningeal deposits and
spinal cor compression are more]Melanoma-10%Gi tract-7%Others-17%