Intraventricular CNS lesions: A pictorial essay

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1 s_s_nner Journl of Medicl Imging nd Rdition Oncology 59 (2015) MEDICAL IMAGING PICTORIAL ESSAY Intrventriculr CNS lesions: A pictoril essy Jne Wtts, 1 Kelvin K Yp, 1 Dniel Ou, 2 Con Trtgli, 1 Nichols Trost 1 nd Tom Sutherlnd 1 1 Deprtment of Medicl Imging, St Vincent s Hospitl, Melourne, Victori, Austrli 2 MIA Blckurn, I-Med, Melourne, Victori, Austrli J Wtts MBBS, FRANZCR; KK Yp MBBS, MMed, FRANZCR, FRACP; DOuMBBS, FRANZCR; C Trtgli MBBS, FRANZCR; N Trost MBBS, FRANZCR; T Sutherlnd MBBS, MMed, FRANZCR. Correspondence Dr Jne Wtts, Medicl Imging Deprtment, 41 Victori Prde, St Vincent s Hospitl, Fitzroy, Vic. 3065, Austrli. Emil: jnewtts@me.com Summry Intrventriculr lesions of the centrl nervous system (CNS) cn present dignostic chllenge due to rnge of differentil dignoses nd rdiologicl ppernces. Both CT nd MRI imging findings, in comintion with loction nd ptient s ge, cn help limit the differentils. This pictoril essy presents the slient rdiologicl fetures, loction nd demogrphics of the more common intrventriculr lesions of the rin. Key words: mgnetic resonnce imging; neurordiology. Conflict of interest: None. Sumitted 29 June 2014; ccepted 26 Jnury doi: / Introduction A vriety of lesions my e identified in the ventriculr system. Knowledge of the common loction of certin lesions (Fig. 1), together with typicl imging chrcteristics (Tle 1) nd presenttion ge (Fig. 2), cn significntly nrrow the differentil dignosis. This pictoril essy serves s reference for the more common intrventriculr lesions of the rin. Bckground The cses in this pictoril essy re derived from n udit of dult crnil MRI studies tht demonstrted intrventriculr lesions with specific histopthologic dignosis t St Vincent s Hospitl, Melourne. One cse, Figure 14, ws cquired t nother institution nd presented to the St Vincent s Medicl Imging Deprtment in clinicordiologicl meeting. Mgnetic resonnce imging studies cquired t St Vincent s Hospitl were performed on 1.5 T Siemens Avnto, 1.5 T Siemens Mgnetom Symphony or 3.0 T Siemens Mgnetom Skyr (Siemens Helthcre, Erlngen, Germny). Conditions Suependymom Suependymoms re rre, enign (World Helth Orgniztion (WHO) grde I), slow-growing, vsculr tumours. They ccount for % of intrcrnil neoplsms, lthough they my e more frequent given tht they re often identified incidentlly in symptomtic ptients. They most commonly occur in middle-ged nd elderly mles. 1 3 The mjority re smll (<2 cm) nd re found in the fourth ventricle ut cn e locted in the lterl ventricle (frontl horn nd ody). On CT, they re well defined, iso/hypottenuting nd loulted with possile clcifiction, hemorrhge nd cystic chnge. On MRI, they re mrkedly T2 hyperintense with T1 iso/hypointensity nd usully do not enhnce (Fig. 3). Histologic nlysis revels fine firillry ckground with occsionl smll, cytologiclly lnd ependymom-like nuclei. Occsionlly, they cn demonstrted fetures of cellulr ependymom in which cse they re clssified s mixed ependymomsuependymom nd grded on the sis of the ependymom component The Royl Austrlin nd New Zelnd College of Rdiologists 453

2 J Wtts et l. Fig. 1. Loction of common intrventriculr lesions. Ependymom Ependymoms (WHO grdes II III) ccount for 3 5% of intrventriculr neoplsms 4 nd occur most frequently in the posterior foss of young ptients. The mjority originte from the floor of fourth ventricle with the remining rising from the lterl nd third ventricles. 1,5 Suprtentoril ependymoms re more frequently intrprenchyml in loction, proly rising from emryonic rest cells, nd re more common in older children nd dults. On CT, they re often hyperttenuting nd heterogeneous secondry to clcifiction (in 50 80%) nd cystic components. Hemorrhge my occur. MRI demonstrtes T1 iso/hypointensity nd T2 hyperintensity with heterogeneous contrst enhncement (Fig. 4) nd vrile Tle 1. Chrcteristics of common intrventriculr lesions Condition T1 T2 Contrst Clcifiction Cystic Hemorrhge Choroid plexus tumour / / / ++ Possile Possile Medullolstom + Possile Possile SEGA / ++ /+ + Ependymom / +/ Possile Centrl neurocytom +/ Rre Colloid cyst Peripherl fint +++ Suependymom / /+ Possile Possile Possile Meningiom + + /+ + Possile Metstsis / / ++ /+ /+ Suependyml gint cell strocytom The Royl Austrlin nd New Zelnd College of Rdiologists

3 Intrventriculr CNS lesions Fig. 2. Age of presenttion of common intrventriculr lesions. looming on T2* weighted imges if clcifiction or hemorrhge re present. They hve propensity for cererospinl fluid (CSF) spred, extending through the formin of Luschk, Mgendie or formen mgnum into the cereellopontine ngles nd cistern mgn respectively. This feture is highly suggestive of n ependymom; however, it is not pthognomonic ecuse medullolstom my occsionlly extend through the formen mgnum. 4 The clssic histologicl fetures of ependymoms re perivsculr pseudorosettes nd true ependyml rosettes, nd the higher grde ependymoms demonstrte incresed cellulrity nd mitotic ctivity. 4 Suependyml gint cell strocytom (SEGA) SEGAs (WHO grde I) represent 1 2% of ll peditric rin tumours, 6 occur in 10 15% of ptients with tuerous sclerosis (TS) 7 nd re the most common cererl neoplsm in this condition. SEGAs re considered pthognomonic for TS; however, there re rre reports of SEGAs in ptients without mnifesttions of TS, thought to e due to somtic mosicism of the TS gene. 4 Typiclly presenting under the ge of 20, lmost ll cses rise ner the formen of Monro. SEGAs re thought to rise from suependyml nodules (SENs), Fig. 3. MRI of 55-yer-old mle ptient with ilterl suependymoms (rrows) of the lterl ventricles. The lesions re difficult to detect on () T2 weighted imges (WI) due to tumour T2 hyperintensity; however, the lesions re demonstrted with () CSF signl suppression on FLAIR imges. (c) Post-gdolinium T1 WI demonstrtes no enhncement. c Fig. 4. MRI of fourth ventriculr ependymom (rrows) in 49-yer-old mle presenting with hedches. The mss is hypointense on T1 WI (), hyperintense on T2 WI () nd demonstrtes moderte enhncement with non-enhncing cystic res on post-gdolinium sgittl T1 WI (c). c 2015 The Royl Austrlin nd New Zelnd College of Rdiologists 455

4 J Wtts et l. Fig. 5. MRI of 28-yer-old femle with tuerous sclerosis (TS). Axil T2 WI () demonstrtes lesions t the formin of Monro (rrows), the right eing prtilly clcified. There re severl hyperintense sucorticl tuers (rrowheds), some which re clcified. Coronl post-gdolinium T1 WI () demonstrtes enhncement of the lesions t the formin of Monro (rrows) consistent with ilterl suependyml gint cell strocytoms (SEGA). supported y rdiologic evidence of seril growth nd y the fct tht they re indistinguishle histologiclly. 8 SEGAs re composed of lrge cells resemling strocytes or gnglion cells with undnt cytoplsm, nd the neoplstic cells my e polygonl, epithelioid or spindle shped. 4,6 Immunohistochemicl testing revels mrkers for oth glil nd neuronl proteins. 4,6 On CT, they re iso/hypottenuting msses (typiclly > 1 cm) tht protrude into the ventricle with vrile clcifiction. On MRI, they re T1 iso/hypointense to gry mtter nd T2 hyperintense or heterogeneous (Fig. 5). Enhncement is vid post contrst dministrtion (Fig. 6). Sucorticl tuers nd SENs re common ssocited findings. The distinction etween SEN nd SEGA is importnt due to the ility of the ltter to grow, ecome symptomtic nd require surgicl mngement. One rticle suggests tht there re certin rdiologicl criteri tht ssist in this distinction. Firstly, the presence of hydrocephlus should define lesion s SEGA, regrdless of its size, position or contrst enhncement, nd secondly, the demonstrtion of growth on seril imging. 8 While evidence suggests the mjority of SEGAs enhnce, mny SENs do so s well. 8 Current recommendtions re for children with dignosis of TS to hve screening MRI performed every 2 3 yers until erly dulthood. Once suependyml lesion hs een dignosed s SEGA or prole SEGA, then mngement should progress to surgery or more ggressive monitoring (vrile reports of nnul to 3 6 monthly MRIs). 4,8 Meningiom Intrventriculr meningioms (WHO grdes I III however usully grde I) re one of the more common intrventriculr neoplsms in the dult popultion, with some studies suggesting they re the most common neoplsm to mnifest in the tri in dulthood. 1 Less commonly, they my rise in the third or rrely the fourth ventricle. They most frequently occur in middleged women with femle-to-mle rtio of 2:1. The ppernce of intrventriculr meningioms on imging is similr to meningioms elsewhere. CT demonstrtes circumscried, slightly hyperttenuting mss with frequent foci of clcifiction (50%). 1 On MRI, they re typiclly T1 nd T2 isointense to grey mtter with intense enhncement (Figs 7,8) tht is usully heterogeneous. Ares of cystic chnge nd necrosis my e present. 9 Intrventriculr meningioms re thought to rise from trpped rchnoid cp cells within the ventricles during development nd the histology of the most common sutype, meningothelil, resemles this. 4 Fig. 6. MRI of 19-yer-old mle with TS presenting with hedche demonstrtes lrge enhncing SEGA on post-gdolinium T1 WI (rrow) cusing ostruction to the left frontl horn. Fig. 7. MRI of 53-yer-old femle with hedches nd vertigo ssocited with n intrventriculr meningiom within the left trium (rrows). The mss is well circumscried, isointense on T1 WI () with homogenous enhncement on post-gdolinium T1 WI () The Royl Austrlin nd New Zelnd College of Rdiologists

5 Intrventriculr CNS lesions Centrl neurocytoms re well-differentited (WHO grde II) neoplsms nd constitute pproximtely % of ll intrcrnil tumours. 1 They occur most frequently in younger nd middle-ged dults. 1,10 Most occur in the frontl horn or ody of the lterl ventricle ner the formen of Monro nd rise from the septum pellucidum or lterl ventriculr wll. Centrl neurocytoms re typiclly wellcircumscried, loulted msses with intrtumourl cysts giving uly ppernce (Fig. 9). On CT, they pper hyperttenuting with frequent foci of clcifiction (in >50%). On MRI, they re heterogeneous, usully T1 isointense to grey mtter nd T2 hyperintense. Prominent flow voids cn e seen. Hemorrhge is rre, nd there is moderte to intense enhncement on postcontrst imging. The histologic fetures of centrl neurocytoms re strikingly similr to oligodendroglioms with oth consisting of uniform round cells with round or ovl nuclei; however, the presence of pineocytomtous rosettes llows the distinction. 4 Similrly, strong stining for synptophysin is considered the most relile dignostic mrker. 4 Choroid plexus tumours (CPTs) Fig. 8. MRI of 49-yer-old-femle with hedches nd lrge right trium meningiom. FLAIR imge demonstrtes circumscried mss tht is predominntly T2 hyperintense with peritumourl oedem nd midline shift. Centrl neurocytom CPTs ccount for 2 4% of peditric rin tumours nd 0.5% of tumours in the dult popultion. They re divided into choroid plexus ppillom (CPP; grde I), typicl CPP (WHO grde II) nd the rrer choroid plexus crcinom (CPC; WHO grde III), which is lmost exclusively found in the peditric popultion. 4 Five to 10% of CPPs degenerte into CPCs. 11 The mjority rise in young children in the tri of the lterl ventricles. They re uncommon in dults where they re typiclly locted in the fourth ventricle. CPTs hve propensity for CSF seeding. CPTs frequently hve loulted, ppillry ppernce. On CT, they re iso or mildly hyperttenuting with possile clcifiction nd cystic chnge. On MRI, CPPs re homogeneously T1 iso/hypointense with vrile T2 signl intensity (Fig. 10). They re vsculr lesions tht enhnce vividly on post-contrst imging. CPCs (Fig. 11) tend to e more heterogeneous ecuse of necrosis, hemorrhge nd cysts; however, differentition etween the different grdes cn e chllenging due to overlpping fetures. Surrounding prenchyml invsion nd peritumourl oedem suggest CPC. 11 CPPs re histologiclly similr to the norml choroid plexus, consisting of cuoidl to columnr epithelil cells surrounding firovsculr stlk. Atypicl CPPs nd CPCs demonstrte progressive incresed cellulrity, mitoses nd nucler pleomorphism. 4 Metstsis Intrventriculr metstses ccount for % of ll cererl metstses. 1 The trium of the lterl ventricle is the most common site for intrventriculr metstsis, followed y the third ventricle. The most common primry tumours in children include neurolstom, Fig. 9. MRI of n 18-yer-old femle with hedche nd ppilloedem due to centrl neurocytom (rrows). T2 WI () nd FLAIR () demonstrte hyperintense, loulted, intrventriculr mss with cystic spces ( uly ppernce ) ttched to the septum pellucidum, with ventriculr dilttion. Post-gdolinium T1WI (c) demonstrtes moderte enhncement. c 2015 The Royl Austrlin nd New Zelnd College of Rdiologists 457

6 J Wtts et l. c Fig. 10. MRI of 20-yer-old mle with choroid plexus ppillom (rrows) of the fourth ventricle. T2 WI () demonstrtes mildly loulted, hyperintense mss with perilesionl oedem extending into the middle cererl peduncles. It is isointense on T1 WI () nd demonstrtes vivid enhncement on post-gdolinium sgittl T1 WI (c). retinolstom nd Wilms tumour nd in dults, melnom, renl, lung (Fig. 12) nd colon crcinom. 1,11 The imging pttern is nonspecific nd my mimic n intrventriculr meningiom or CPP; however, specific chrcteristics of some tumours cn nrrow the differentil, such s T1 hyperintensity in melnom. Typiclly there is vid enhncement fter contrst dministrtion nd vsogenic oedem my e seen in the djcent rin prenchym. 4 Colloid cyst Colloid cysts re the most common lesion in the third ventricle; however, they re rre nd occurring in pproximtely three per million people per yer. 12 They my e detected incidentlly on imging, with hedches (occurring in % of ptients 12 nd often the presenting symptom) or with cute hydrocephlus tht my e ftl (most common in the third to fifth decde). 13 Colloid cysts re found in the nterosuperior spect of the third ventricle ner the formen of Monro nd re round or ovoid in shpe. The loction nd imging chrcteristics re virtully pthognomonic. Colloid cysts re typiclly hyperttenuting on CT with respect to the rin due to intrinsic elevted protein nd cn vry from few millimetres to 3 cm. On MRI, they hve vrile ppernce. Approximtely 50% re hyperintense on T1-weighted imges with respect to rin nd on T2-weighted sequences most cysts re hypointense (Fig. 13). 12 They my e difficult to detect on FLAIR if T2 hypointense. Intrcystic fluid levels my e seen, nd thin rim of enhncement my e present indicting the cyst cpsule. Clcifiction is exceptionlly rre. Histologiclly, colloid cysts re chrcterised y simple to pseudostrtified epithelil lining with scttered mucous golet nd cilited cells. When detected rdiologiclly, it is importnt tht the finding of colloid cyst is promptly relyed to the referrer ecuse of the risk of cute ostructing hydrocephlus, prticulrly given tht cyst size is not relile predictor of clinicl outcome. 12 Medullolstom Medullolstoms (WHO grde IV) re the most common posterior foss tumour in children (mles > femles) (Fig. 14), representing 12 25% of ll centrl nervous system tumours in the peditric popultion ut only Fig. 11. MRI of 56-yer-old femle with ilterl ppilloedem due to choroid plexus crcinom (rrows). T2 WI () demonstrtes n irregulr, loulted, isointense mss in the left trium. Post-gdolinium T1 WI () demonstrtes intense enhncement with irregulr infiltrtive mrgins. The ppernce of this lesion is similr to the choroid plexus ppillom in Figure 9. Fig. 12. MRI of n 82-yer-old mle with txi nd metsttic non-smll-cell lung cncer demonstrtes well-defined, isointense mss in the right trium (rrow) on T2 WI () with homogeneous, intense enhncement on postgdolinium T1 WI () The Royl Austrlin nd New Zelnd College of Rdiologists

7 Intrventriculr CNS lesions Fig. 13. MRI of 56-yer-old womn with n incidentl third ventriculr colloid cyst. T1 WI () demonstrtes n ovoid mss t the formen of Monro tht is hyperintense to CSF nd hypointense on T2 WI (). On post-gdolinium T1 WI (c) fine, peripherl septl veins re visile. c 0.4 1% in dults. 14 The overll men ge of presenttion for ll ge groups is 13 yers nd in dults typiclly presents etween 20 nd 40 yers. This highly mlignnt neoplsm hs propensity to CSF spred. In children, they most frequently rise from the roof nd project into the fourth ventricle. In dults, they rise in the cereellr hemisphere. On CT, medullolstoms re well-circumscried, homogeneous, hyperttenuting with enhncement nd demonstrte vrile clcifiction nd cyst formtion, 59% nd 22%, respectively, in one study. 14 On MRI, they re T1 hypointense to grey mtter with vrile ppernce on T2-weighted sequences, ut the solid component is typiclly iso to hyperintense. Heterogeneous enhncement fter contrst is usul, nd mrginl vsogenic oedem is common. Histologiclly, there re four mjor sutypes with the clssic sutype defined y dense, sheet-like growth of cells with hyperchromtic round-to-ovl nuclei. Neurolstic or Homer- Wright rosettes re common fetures. Conclusion Ptient ge, lesion loction nd imging findings together cn ssist to nrrow the differentil dignosis of Fig month-old with fourth ventriculr medullolstom who presented with drowsiness. CT () demonstrtes hyperttenuting mss (rrow) effcing the fourth ventricle with surrounding oedem. Sgittl postgdolinium T1 WI MRI () demonstrtes extensive enhncing leptomeningel spred (rrows). intrventriculr lesions in n otherwise heterogeneous group of conditions. References 1. Koeller KK, Snderg GD, Armed Forces Institute of Pthology. From the rchives of the AFIP. Cererl intrventriculr neoplsms: rdiologicpthologic correltion. Rdiogrphics 2002; 22: Wldron JS, Tihn T. Epidemiology nd pthology of intrventriculr tumors. Neurosurg Clin N Am 2003; 14: Rgel BT, Osorn AG, Whng K, Townsend JJ, Jensen RL, Couldwell WT. Suependymoms: n nlysis of clinicl nd imging fetures. Neurosurgery 2006; 58: Boyd Smith A, Smirniotopoulos JG, Horknyne-Szkly I. From the rdiologic pthology rchives. Intrventriculr neoplsms: rdiologicpthologic correltion. Rdiogrphics 2013; 33: Dumont AS, Frce E, Schiff D, Shffrey ME. Intrventriculr glioms. Neurosurg Clin N Am 2003; 14: Grjkowsk W, Kotulsk K, Jurkiewicz E et l. Suependyml gint cell strocytoms with typicl histologicl fetures mimicking mlignnt glioms. Foli Neuropthol 2011; 49: Jozwik S, Nout R, Curtolo P, Epilepsy M, prticipnts of the TSCCMfS. Mngement of suependyml gint cell strocytom (SEGA) ssocited with tuerous sclerosis complex (TSC): clinicl recommendtions. Eur J Peditr Neurol 2013; 17: Goh S, Butler W, Thiele EA. Suependyml gint cell tumors in tuerous sclerosis complex. Neurology 2004; 63: Mjos C, Cucurell G, Aguiler C, Coll S, Pons L. Intrventriculr meningioms: MR imging nd MR spectroscopic findings in two cses. AJNR Am J Neurordiol 1999; 20: Lee J, Chng SM, McDermott MW, Prs AT. Intrventriculr neurocytoms. Neurosurg Clin N Am 2003; 14: The Royl Austrlin nd New Zelnd College of Rdiologists 459

8 J Wtts et l. 11. Neini RM, Yoo JH, Hunter JV. Spectrum of choroid plexus lesions in children. AJR Am J Roentgenol 2009; 192: Armo D, Cstillo M, Chen H, Kwock L. Colloid cyst of the third ventricle: imging pthologic correltion. AJNR Am J Neurordiol 2000; 21: Mjos C, Coll S, Aguiler C, Acees JJ, Pons LC. Intrventriculr mss lesions of the rin. Eur Rdiol 2000; 10: Koeller KK, Rushing EJ. From the rchives of the AFIP: medullolstom: comprehensive review with rdiologic-pthologic correltion. Rdiogrphics 2003; 23: The Royl Austrlin nd New Zelnd College of Rdiologists

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