cns pathology third year medical students · 2018-08-11 · cns lymphoma • aggressive disease...
TRANSCRIPT
CNSpathologyThirdyearmedicalstudents
DrHeyamAwad2018
Lecture13:CNStumours3/3
meningioma
•Arisefromarachnoidmeningothelialcells.•Ariseinadults•Attachedtothedura•Canbeseenatexternalsurfacesofthebrainorwithintheventricularsystem
meningioma
•Majority:canbeeasilyseparatedfrombrain,butsomeareinfiltrative•Behaviour:benignbutinfiltrativelesionsrecur•Outcomedependson:size,location,histologicalgrade
Histologicalgrades
•WHOI:(welldiff)meningioma•WHOII:atypicalmeningioma•WHOIII:anaplastic(malignant)meningioma
Grade1meningiomas
•Welldefined,durabasedmasses•Maycompressbutdonotinvadebrain•Canextendtooverlyingbone
• Grade1meningiomas/histologicaltypes• •Syncytial:whorledclusterswithoutvisiblecellmembranes.• •Fibroblastic:elongatedcellsandabundantcollagen• •Transitional:featuresofboth,syncytialandfibroblastic• •Psammomatous:numerouspsammomabodies
syncitial
fibroblastic
pasammomatous
AtypicalmeningiomaWHOgrade2
•Highcellularity•Prominentnuclei•Highmitoticrate•Moreaggressivethangrade1•Recur
Atypicalmeningioma
Anaplasticmeningioma
• AnaplasticmeningiomaWHOgrade3•Highlyaggressive•Resemblesarcomas
PrimaryCNSlymphoma
• Mostly:diffuselargeBcelllymphoma.•1%ofintracranialtumors.•PrimaryCNSlymphomaisthemostcommonCNSneoplasmintheimmunocompromised…inthissituationtheyarealmostalwayspositiveforEBV((Epstein– Barrvirus)
CNSlymphoma
• Aggressivediseasewithpoorprognosis•Poorresponsetochemotherapyascomparedtoperipherallymphomas•Usuallymultiplenoduleswithinthebrainparenchyma•Spreadingoutsidethebrainhappensrarelyandatlatestages•Peripherallymphomararelyspreadstothebrain,ifitdoesthereisusuallyassociatedmeningealandCNSinvolvement.
lymphoma
• Involvesdeepgreymatter,whitematter,cortex•Periventricularspreadiscommon•Tumornodulesmoredefinedthangliomasbutlessthanmetastases•EBVpositivetumorsusuallyhaveextensiveareasofnecrosis•Majority:diffuselargeBcelllymphomas
lymphoma:notethemultiplicityofthelesions
lymphoma
Metastatictumors
- ¼to½ofintracranialtumors•Mostcommonprimarysites:lung,breast,melanoma,kidneyandGIT.•Formdiscretewelldefinedmasses,canbemultiple
Paraneoplasticsyndromes
•CNSandperipheralnervescanbeaffectedindisseminatedcanceraspartoftheparaneoplasticsyndromes•Theseincludeseveralmanifestationsincludingdementia,ataxia,sensoryneuropathyandpsychosis
Familialtumorsyndromes
•Inheritedsyndromes•Mutationsinseveraltumorsuppressorgenes•Associatedwithincreasedriskofcertaintypesofcancer•2syndromeswithCNSinvolvement:TuberoussclerosisandvonHippel- Lindou
•Tuberoussclerosis
• Autosomaldominant• Hamartmasandbenignneoplasmsinbrainandothersites• CNStumors:corticaltubersandsubependymalhamartomas
• Tuberoussclerosis
•Corticaltubers:looklikepotatoes!!•Tuber:thickenedundergroundpartofastem
• Corticaltubers:Hamartomascomposedofhaphazardlyarrangedlargeneurones.• Mixtureofglialandneuronalcells• Causeseizures
Tuberoussclerosis..potatoesinthebrain!
• Subependymaltubers:Similartocorticaltubers• Cancausehydrocephalus
• Tuberoussclerosis/Extracerebrallesions
• renalangiomyolipoma,• retinalglialhamartomas,• pulmonarylymphangiomatosis• cardiacrhabdomyoma• cystsinliver,kidney,pancreas.• skinlesions:angiofibroma,hypopigmentedareas,thichenedpatches.
•VonHippelLindausyndrome
• Autosomaldominant• MutationinVHLtumorsupressorgene.• Hemangioblastomasmainlyincerebellarhemispheres,retina.• Cystsinpancreas,liverkidney• Increaseriskofrenalcellcarcinoma