Tumors of the CNS


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  • Less than 5 years small round cell tumors eg wills, neuroblastoma leukemia's ets between 5 to 14 lymphomas and CNS tumors
  • Upto grade 2 surgery is enough after radio therapy must be added
  • Optic glioma
  • NB fibrially back ground
  • Hypercellularity + nuclear atypia
  • Increased cellularity + nuclear atypia+ mitotic figures
  • Grade + giant cells
  • Pseudo pallisding central necrosis with perpendicular cells
  • Easily removed with little neurological complication rarely
  • USAULLY bilaterally
  • Tumor material different from brain tissue
  • Tumors of the CNS

    1. 1. Central Nervous SystemCentral Nervous System 9.9. TumorsTumors Central Nervous SystemCentral Nervous System 9.9. TumorsTumors
    2. 2. INTRACRANIAL TUMORS (ICTS)INTRACRANIAL TUMORS (ICTS) Primary or MetastaticPrimary or Metastatic Occur withOccur with equal frequency in adultsequal frequency in adults, but in, but in children primarychildren primary tumors are far more common.tumors are far more common. Primary ICTs account forPrimary ICTs account for – ~2% of cancers in adults~2% of cancers in adults – 20% of all cancers in children.20% of all cancers in children. InIn children 70% of ICTs arise inchildren 70% of ICTs arise in  Posterior fossa (infra-Posterior fossa (infra- tentorial).tentorial). asas InIn adultsadults 70% of ICTs arise in70% of ICTs arise in  Supra-tentorialSupra-tentorial.. Because of its location, aBecause of its location, a benign ICTbenign ICT may have fatalmay have fatal “malignant” effects“malignant” effects.. Malignant ICTs spread by:Malignant ICTs spread by: – Direct infiltration of adjacent tissuesDirect infiltration of adjacent tissues – May disseminate within the CNS via CSF.May disseminate within the CNS via CSF. GliomasGliomas account foraccount for 60% of primary ICTs60% of primary ICTs Meningiomas for 20% & all others 20%.Meningiomas for 20% & all others 20%. All CNS tumors behave as malignant clinically . Limited spaceAll CNS tumors behave as malignant clinically . Limited space
    3. 3. The unique features of CNS tumors – “ICP” 1. CNS tumors- < 2% of all malignant tumors. They grow in a unique environment: the intracranial space. 2. The intracranial contents - incompressible Brain and blood contained within a rigid unyielding bony structure. 3. Intracranial pathologies (tumors, abscess, hematoma, infarction, edema, etc.) eventually produce life threatening increase of the intracranial-pressure: ICP.
    4. 4. Cytologic origin of CNS tumorsCytologic origin of CNS tumors Neuro-ectodermalNeuro-ectodermal – most important are the– most important are the GliomasGliomas;; MesenchymalMesenchymal – most frequent ones are the– most frequent ones are the MeningiomasMeningiomas;; Ectopic tissuesEctopic tissues – from tissues „displaced” during– from tissues „displaced” during embryogenesis: Ex.,embryogenesis: Ex., DermoidDermoid cyst;cyst; Retained embryonal structuresRetained embryonal structures: various cysts –: various cysts – Paraphyseal cystParaphyseal cyst;; MetastasesMetastases:: Lung, Breast, Melanoma, etc. in 50%Lung, Breast, Melanoma, etc. in 50% of casesof cases
    5. 5. Neuro – ecto - dermal tumorsNeuro – ecto - dermal tumors Glial cells:Glial cells: astrocytes (A) -astrocytes (A) - AstrocytomaAstrocytoma Oligodendroglial cells -Oligodendroglial cells - OligodendrogliomaOligodendroglioma Ependymal cells –Ependymal cells – EpendymomaEpendymoma Neurons -Neurons - GangliocytomaGangliocytoma
    6. 6. Incidence of brain tumorsIncidence of brain tumors
    7. 7. Seen in kids with increased cellularity
    8. 8. What is „benign” and „malignant” in case of CNS tumors?
    9. 9. ASTROCYTOMASASTROCYTOMAS Account for ~Account for ~ 80% of primary ICTS in adults80% of primary ICTS in adults MC in theMC in the cerebral hemispherescerebral hemispheres MC Symptoms: headaches, seizures, focalMC Symptoms: headaches, seizures, focal neurologic deficits ( usually in the anterior orneurologic deficits ( usually in the anterior or middle)middle) Low-grade AstrocytomasLow-grade Astrocytomas:: – Gross:Gross: Poorly defined gray-white infiltrative tumors.Poorly defined gray-white infiltrative tumors. – Histology:Histology: HypercellularityHypercellularity; astrocytic nuclei of mild degree of atypia &; astrocytic nuclei of mild degree of atypia & astrocytic processesastrocytic processes ⇒⇒ fibrillary background = fingers offibrillary background = fingers of astrocytesastrocytes
    10. 10. Low-grade AstrocytomasLow-grade Astrocytomas Pilocytic AstrocytomasPilocytic Astrocytomas:: – MC in theMC in the cerebellum of children & young adultscerebellum of children & young adults; and; and less commonly in theless commonly in the optic nerveoptic nerve, hypothalamic, hypothalamic region or cerebral hemispheresregion or cerebral hemispheres – Morphology:Morphology: Cystic, with a tumor nodule in the wall of the cyst.Cystic, with a tumor nodule in the wall of the cyst. Composed of bipolar astrocytes, with long hair-likeComposed of bipolar astrocytes, with long hair-like processes,processes, Rosenthal fibersRosenthal fibers && Micro-cysts+Micro-cysts+ calcification = good prognosiscalcification = good prognosis – Grow very slowly (some patients have survived forGrow very slowly (some patients have survived for >40 yrs after incomplete resection) & have an>40 yrs after incomplete resection) & have an Excellent prognosisExcellent prognosis – DD ;- not to confuse with low grade FibrillaryDD ;- not to confuse with low grade Fibrillary AstrocytomaAstrocytoma
    11. 11. Grade I. tumor: pilocytic astro~Grade I. tumor: pilocytic astro~
    12. 12. Pilocytic AstrocytomaPilocytic Astrocytoma
    13. 13. Pilocytic AstrocytomaPilocytic Astrocytoma
    14. 14. Pilocytic AstrocytomaPilocytic Astrocytoma
    15. 15. Rosenthal fibersRosenthal fibers
    16. 16. Gr. II. AstrocytomaGr. II. Astrocytoma
    17. 17. Gr. II. astrocytomaGr. II. astrocytoma
    18. 18. Gr. III. astrocytomaGr. III. astrocytoma
    19. 19. Gr. III. astrocytomaGr. III. astrocytoma
    20. 20. GGrr. III. Astrocytoma. III. Astrocytoma
    21. 21. Gr. IV. astro ~ = GBMGr. IV. astro ~ = GBM
    22. 22. Gr. IV. astro ~ = GBMGr. IV. astro ~ = GBM
    23. 23. Gr. IV. astro ~ = GBMGr. IV. astro ~ = GBM
    24. 24. GBM: necrosis/pseudo-palisadeGBM: necrosis/pseudo-palisade
    25. 25. GBM: pleomorphic cytologyGBM: pleomorphic cytology
    26. 26. Gr. IV. Astro ~ = GBMGr. IV. Astro ~ = GBM Die directly
    27. 27. OLIGODENDROGLIOMAOLIGODENDROGLIOMA Comprise ~ 5 -15% of GliomasComprise ~ 5 -15% of Gliomas Arise in theArise in the cerebral white mattercerebral white matter MC in theMC in the 4th & 5th decades4th & 5th decades Gross:Gross: – Well circumscribed, gelatinous, gray masses, withWell circumscribed, gelatinous, gray masses, with foci offoci of hemorrhage & calcification.hemorrhage & calcification. Histology:Histology: – Sheets of cells with rounded nuclei surrounded by a haloSheets of cells with rounded nuclei surrounded by a halo of clear cytoplasm (of clear cytoplasm (fried egg appearancefried egg appearance).). – There is often a delicate network of capillaries &There is often a delicate network of capillaries & scattered foci of calcification (scattered foci of calcification (psammoma bodiespsammoma bodies)( seen)( seen in thyroid, CNS, kidneys etc).in thyroid, CNS, kidneys etc). Grows slowly, presents commonly with seizures,Grows slowly, presents commonly with seizures, prognosis is better than Astrocytomaprognosis is better than Astrocytoma, average, average survival is 5-10 yrs (with modern therapeuticsurvival is 5-10 yrs (with modern therapeutic approachesapproaches
    28. 28. OligodendrogliomaOligodendroglioma
    29. 29. OligodendrogliomaOligodendroglioma
    30. 30. OligodendrogliomaOligodendroglioma
    31. 31. Oligo-AstrocytomaOligo-Astrocytoma
    32. 32. EPENDYMOMAEPENDYMOMA Arise from theArise from the Ependymal liningEpendymal lining of the ventricles orof the ventricles or the central canal of the spinal cordthe central canal of the spinal cord Arise in theArise in the – Fourth ventricleFourth ventricle inin children & young adultschildren & young adults – Spinal cord in the middle aged.Spinal cord in the middle aged. Morphology:Morphology: – Highly cellular, tumor cells have regular nucleiHighly cellular, tumor cells have regular nuclei – May exhibit epithelial features with formation of “May exhibit epithelial features with formation of “rosettesrosettes”” (Flexner…) or “canals”, also perivascular(Flexner…) or “canals”, also perivascular pseudo-pseudo- rosettes (homer …)rosettes (homer …) – Most tumors areMost tumors are well differentiatedwell differentiated 4th ventricle tumors:4th ventricle tumors: – May cause hydrocephalus, usually can’t be completelyMay cause hydrocephalus, usually can’t be completely removedremoved CSF dissemination may occurCSF dissemination may occur Average survival is ~ 4 yrsAverage survival is ~ 4 yrs
    33. 33. Myxo-papillary EpendymomasMyxo-papillary Ependymomas – Arise in theArise in the filum terminalefilum terminale of the spinal cordof the spinal cord – Prognosis depends on completeness ofPrognosis depends on completeness of surgical excisionsurgical excision
    34. 34. EpendymomaEpendymoma ““Rosettes” & perivascular Pseudo-rosettesRosettes” & perivascular Pseudo-rosettes
    35. 35. MEDULLOBLASTOMASMEDULLOBLASTOMAS Second MC ICT of childhood (after Astrocytomas).Second MC ICT of childhood (after Astrocytomas). OccursOccurs exclusivelyexclusively in thein the cerebellumcerebellum.. Derived from fetalDerived from fetal external granular layer ofexternal granular layer of cerebellum.cerebellum. Grows rapidly & occludes CSF flowGrows rapidly & occludes CSF flow →→ hydrocephalus.hydrocephalus. Seeds through CSFSeeds through CSF →→ implants around the spinalimplants around the spinal cord & cauda equina (need irradiation of the wholecord & cauda equina (need irradiation of the whole Neuraxis).Neuraxis). Histology:Histology: – Extremely cellular, anaplastic, small round orExtremely cellular, anaplastic, small round or carrot-shapedcarrot-shaped cellscells with hyperchromatic nuclei,with hyperchromatic nuclei, ↑↑ N/C, may formN/C, may form Homer-Homer- Wright pseudo-rosettesWright pseudo-rosettes Highly malignantHighly malignant, yet, yet radiosensitiveradiosensitive & 5-yr survival& 5-yr survival 75%.75%.
    36. 36. MedulloblastomaMedulloblastoma
    37. 37. MedulloblastomaMedulloblastoma Homer-Wright pseudo-rosettesHomer-Wright pseudo-rosettes carrot-shaped cellscarrot-shaped cells
    38. 38. MENINGIOMASMENINGIOMAS UsuallyUsually Benign slow-growingBenign slow-growing tumors of adults, F/M 3:2tumors of adults, F/M 3:2 Originate fromOriginate from meningothelial cellsmeningothelial cells of the arachnoid.of the arachnoid. UsuallyUsually solitarysolitary ( multiple meningiomas( multiple meningiomas ⇒⇒ NF2 )NF2 ) Morphology:Morphology: – Firm rounded masses, adherent to the dura andFirm rounded masses, adherent to the dura and compressing the underlying brain (no infiltration).compressing the underlying brain (no infiltration). – Histologic variants include:Histologic variants include: Syncytial, fibroblastic, transitional, Psammomatous &Syncytial, fibroblastic, transitional, Psammomatous & papillary (papillary (↑↑ propensity to recur).propensity to recur). Malignant Meningioma is very rareMalignant Meningioma is very rare – Infiltrates the underlying brain, shows marked nuclear atypia,Infiltrates the underlying brain, shows marked nuclear atypia, ↑↑ mitoses, & foci of necrosis.mitoses, & foci of necrosis. Other rare sarcomas of meninges include:Other rare sarcomas of meninges include: – Hemangiopericytoma, malignant fibrous histiocytoma &Hemangiopericytoma, malignant fibrous histiocytoma & Fibrosarcoma.Fibrosarcoma.
    39. 39. MeningiomaMeningioma
    40. 40. MeningiomaMeningioma SyncytialSyncytial PsammomatousPsammomatous Epithelial Membrane Antigen
    41. 41. NERVE SHEATH TUMORSNERVE SHEATH TUMORS 1. Schwannomas1. Schwannomas:: – BenignBenign tumors of Schwann cellstumors of Schwann cells – MC in theMC in the vestibular branch of the VIII CNvestibular branch of the VIII CN at the cerebello-at the cerebello- pontine angle (acoustic neuroma)pontine angle (acoustic neuroma) →→ tinnitus & hearing losstinnitus & hearing loss – Also involve branches of the trigeminal nerve & dorsal nerveAlso involve branches of the trigeminal nerve & dorsal nerve rootsroots – Tumors are encapsulated, attached to one side of the nerve;Tumors are encapsulated, attached to one side of the nerve; axons do not pass through the tumoraxons do not pass through the tumor Consist ofConsist of Antoni -AAntoni -A areas of high cellularityareas of high cellularity – Nuclei form palisades “Verocay bodies”Nuclei form palisades “Verocay bodies” Antoni -BAntoni -B myxoid areasmyxoid areas
    42. 42. SchwannomaSchwannoma Antoni – A hyprecellularAntoni – A hyprecellular Antoni –B Sparsely cellularAntoni –B Sparsely cellular
    43. 43. 2. Neurofibromas2. Neurofibromas:: – BenignBenign tumors composed predominately of Schwann cells, but alsotumors composed predominately of Schwann cells, but also containing fibroblasts & perineural cellscontaining fibroblasts & perineural cells – May involve single or multiple dorsal spinal nerve roots (May involve single or multiple dorsal spinal nerve roots (multiplemultiple inin patients withpatients with von Ricklinghausen's disease - NF1von Ricklinghausen's disease - NF1)) – CN involvement is extremely rareCN involvement is extremely rare – May present asMay present as Localized fusiform enlargement of a nerve orLocalized fusiform enlargement of a nerve or Extensively infiltrate along the nerveExtensively infiltrate along the nerve →→ “ropy enlargement”“ropy enlargement” of theof the nerve & it’s branchesnerve & it’s branches (plexiform Neurofibroma)(plexiform Neurofibroma) – PlexiformPlexiform neurofibromas are usually part ofneurofibromas are usually part of NF1NF1, excision is very, excision is very difficultdifficult – Histology:Histology: Wavy spindle shaped cells, myxoid collagenous stroma withWavy spindle shaped cells, myxoid collagenous stroma with interspersed nerve fibersinterspersed nerve fibers
    44. 44. „„Acoustic Neurinoma”Acoustic Neurinoma” (Schwannoma)(Schwannoma)
    45. 45. METASTATIC ICTSMETASTATIC ICTS 50% of ICTs.50% of ICTs. Common Primaries:Common Primaries: – Broncho-genic small cell undifferentiated (oat cell) ca.,Broncho-genic small cell undifferentiated (oat cell) ca., Breast ca., Malignant melanoma, RCC. & Colon ca.Breast ca., Malignant melanoma, RCC. & Colon ca. Sites of metastases:Sites of metastases: – Cerebral cortex 80%;Cerebral cortex 80%; – Rest are in the cerebellum & brain stem.Rest are in the cerebellum & brain stem. 50% are multiple50% are multiple; at the; at the junctionjunction between thebetween the gray &gray & white matter.white matter. Vertebral columnVertebral column is a common site for metastasesis a common site for metastases ofof – BreastBreast && ProstaticProstatic carcinomascarcinomas – ThoracicThoracic spine 60%, Cervical 20% & Lumbar 20%spine 60%, Cervical 20% & Lumbar 20% Treatment : RadiotherapyTreatment : Radiotherapy
    46. 46. MetastasisMetastasis
    47. 47. CHOROID PLEXUS PAPILLOMACHOROID PLEXUS PAPILLOMA MC in childrenMC in children  Arising from theArising from the laterallateral ventriclesventricles InIn adultsadults they are found MC in thethey are found MC in the 4th4th ventricleventricle Present withPresent with HydrocephalusHydrocephalus – Due to either over-production of CSF or toDue to either over-production of CSF or to obstruction of the ventricular system.obstruction of the ventricular system. Consist ofConsist of papillae with fibrovascularpapillae with fibrovascular stalksstalks covered with a cuboidal or columnarcovered with a cuboidal or columnar ciliated epithelium, recapitulating theciliated epithelium, recapitulating the structure of the normal choroid plexus.structure of the normal choroid plexus.
    48. 48. COLLOID CYST OF THE THIRD VENTRICLECOLLOID CYST OF THE THIRD VENTRICLE AA non-neoplastic cysticnon-neoplastic cystic lesionlesion Morphology:Morphology: – Having a thin fibrous capsule, a lining ofHaving a thin fibrous capsule, a lining of Cuboidal toCuboidal to columnarcolumnar epithelium & containing gelatinousepithelium & containing gelatinous Proteinaceous material.Proteinaceous material. Attached to the roof of theAttached to the roof of the third ventriclethird ventricle at theat the foramina of Munroe & may cause suddenforamina of Munroe & may cause sudden obstruction of the CSF flowobstruction of the CSF flow →→ acute non-acute non- communicating hydrocephaluscommunicating hydrocephalus →→ brainbrain herniation & deathherniation & death Symptoms: headaches (often positional),Symptoms: headaches (often positional), “drop“drop attacksattacks”, incontinence”, incontinence Goblet cellsGoblet cells are confirmatoryare confirmatory
    49. 49. MISCELLANEOUS (MISCELLANEOUS (MIDLINEMIDLINE) TUMORS) TUMORS PinealomasPinealomas:: – True pineocytomas areTrue pineocytomas are extremely rareextremely rare, may also have, may also have pineoblastomaspineoblastomas GerminomasGerminomas:: – MC in the pineal & suprasellar regions inMC in the pineal & suprasellar regions in adolescents &adolescents & young adultsyoung adults – Closely resemble testicular Seminomas & ovarianClosely resemble testicular Seminomas & ovarian DysgerminomasDysgerminomas – Other GCTs (Teratomas & Choriocarcinomas) also occurOther GCTs (Teratomas & Choriocarcinomas) also occur Not clear how GCTs arise within the CNSNot clear how GCTs arise within the CNS CraniopharyngiomasCraniopharyngiomas:: – Benign cystic tumors of children & adolescentsBenign cystic tumors of children & adolescents – Develop in theDevelop in the suprasellarsuprasellar regionregion →→ HypopituitarismHypopituitarism – Originate from remnants ofOriginate from remnants of Rathke’s pouchRathke’s pouch & contain& contain squamous & columnar epithelium,squamous & columnar epithelium, calcificationscalcifications areare common.common.
    50. 50. CNS LYMPHOMACNS LYMPHOMA PrimaryPrimary CNS lymphomasCNS lymphomas – Account forAccount for ~1%~1% of ICTsof ICTs – MC CNS neoplasm inMC CNS neoplasm in AIDSAIDS & other& other immunosuppressedimmunosuppressed patients often arise deep withinpatients often arise deep within thethe cerebral hemispherescerebral hemispheres & are commonly& are commonly bilateralbilateral – Lymphoma cells exhibit an angiocentric distributionLymphoma cells exhibit an angiocentric distribution – Usually areUsually are B-cellB-cell lymphomas & many appear to belymphomas & many appear to be EBV-related.EBV-related. SecondarySecondary CNS lymphomasCNS lymphomas – Lymphomas arising outside of the CNSLymphomas arising outside of the CNS rarely involverarely involve the brain parenchymathe brain parenchyma – May involve the meninges, intradural spinal nerveMay involve the meninges, intradural spinal nerve roots & epidural spaceroots & epidural space
    51. 51. PHAKOMATOSESPHAKOMATOSES NEUROCUTANEOUS SYNDROMESNEUROCUTANEOUS SYNDROMES (PHAKOMATOSES)(PHAKOMATOSES) ADAD Hamartomas & NeoplasmsHamartomas & Neoplasms – Esp. involving theEsp. involving the nervous system & skinnervous system & skin – Mutations in tumor suppressor genesMutations in tumor suppressor genes 1.1. Neurofibromatosis Type 1 (Neurofibromatosis Type 1 (NF1NF1)):: – Neurofibromas, Neurofibro-sarcomasNeurofibromas, Neurofibro-sarcomas – Optic nerve GliomasOptic nerve Gliomas – Pigmented cutaneous macules (café au lait spots)Pigmented cutaneous macules (café au lait spots) – Pigmented nodules of iris (Pigmented nodules of iris (Lisch nodulesLisch nodules)) 2.2. Neurofibromatosis Type 2 (Neurofibromatosis Type 2 (NF2NF2)):: – Bilateral Schwannomas of CN VIIIBilateral Schwannomas of CN VIII – Multiple meningiomasMultiple meningiomas – Spinal cord EpendymomasSpinal cord Ependymomas
    52. 52. 3.3. Tuberous SclerosisTuberous Sclerosis HamartomasHamartomas (“tubers”) in the cerebral(“tubers”) in the cerebral cortex, Sub-Ependymal hamartomascortex, Sub-Ependymal hamartomas (“candle drippings”)(“candle drippings”) ⇒⇒ Sub-EpendymalSub-Ependymal giant cell Astrocytomasgiant cell Astrocytomas Seizures & mental retardationSeizures & mental retardation Extra CNSExtra CNS findings:findings: – Kidney (Angiomyolipoma), HeartKidney (Angiomyolipoma), Heart (Rhabdomyoma MCC in kids, adult =(Rhabdomyoma MCC in kids, adult = mixomas ), skin (Angiofibroma)mixomas ), skin (Angiofibroma)
    53. 53. Tuberous SclerosisTuberous Sclerosis
    54. 54. 4.4. von Hippel-Lindau diseasevon Hippel-Lindau disease HemangioblastomasHemangioblastomas of the cerebellum,of the cerebellum, retina, brain stem & spinal cordretina, brain stem & spinal cord CystsCysts of liver, kidney & pancreasof liver, kidney & pancreas ↑↑↑↑ incidence ofincidence of RCCRCC, may be bilateral, may be bilateral ~ 10% of Hemangioblastomas~ 10% of Hemangioblastomas ⇒⇒ polycythemiapolycythemia
    55. 55. von Hippel-Lindau diseasevon Hippel-Lindau disease HemangioblastomasHemangioblastomas