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Home , Central neurocytoma, Ependymoma, Meningioma, Neurocytoma, Subependymoma

Intraventricular tumours

• • Intraventricular tumours • Papilloma/Ca Subependymal Giant Tumour/ (SEGA) Sumeet Kumar • National Neuroscience Institute • Duke-NUS Medical School • Central Singapore • Others- GBM, , RGNT (Rosette forming Glioneuronal Tumour), Metastases

Ependymoma Cells of origin of intraventricular tumours Ependymal cells lining the ventricles Arise from epithelial Epithelial lining of ventricles- • • → ependymoma lining of ventricles- • Adjacent to ependymal lining- subependymal plate of glial cells → ependyma subependymoma

• Choroid plexus → choroid plexus tumours- papilloma, atypical papilloma, carcinoma • Vascularity of choroid plexus → metastases • Parenchymal • Arachnoidal cap cells which form arachnoid granulations can be arise trapped in the choroid plexus → meningioma from embryologically • Septum pellucidum lined by glial cells and neuronal precursor cells → trapped ependymal cells

http://missinglink.ucsf.edu/lm/introductionneuropathology

Ependymoma Ependymoma

• 3-5% of intracranial • More common in • Gross pathology- soft children (1-6 years) “plastic” tumours, • 60% posterior fossa In the posterior fossa “toothpaste”tumours th • 40% supratentorial • Supratentorial lesions- • 4 ventricular tumours extend through foramen • Half parenchymal and mean age 18-24 years half intraventricular of Luschka into CP Angle • Children have a worse or through foramen prognosis- higher magnum prevalence of anaplastic form • - perivascular rosettes and true ependymal rosettes

Osborn. Copyright Amirsys Inc 2004 http://missinglink.ucsf.edu/lm/introductionneuropathology Ependymoma 47/F 4th ventricular ependymoma with Ependymoma 47/F 4th ventricular ependymoma with hydrocephalus

Fill 4th ventricle like plaster cast Cystic components Fill 4th ventricle like plaster cast frequent Intraventricular ependymomas can extend Can seed through CSF (12%) Extend thru the foramen of Luschka and Calcification into adjacent parenchyma Extend thru the foramen of Luschka and - NOT PATHOGNOMIC- Hemorrhage foramen magnum- NOT PATHOGNOMIC- Imaging of entire neuroaxis occasionally also Mildly reduced occasionally medulloblastomas also diffusion

Subependymoma Subependymoma

• 0.2- 0.7 % of intracranial • WHO grade 1 neoplasms Mostly incidental • • Ependymal • 50-60% in 4th ventricle differentiation • 30-40% in • Well circumscribed, • M:F= 2.3:1 attached to the ventricle • Most patients in 5th and wall by a narrow pedicle 6th decades • Prognosis- good

Osborn. Copyright Amirsys Inc 2004

Subependymoma Choroid Plexus Tumours

• Well circumscribed • 2-4% of pediatric brain tumours tumours • Most commonly child <5 • Cystic change common years of age • Mostly no/ minimal • 20% of brain tumours enhancement occurring in the first year of life are Choroid plexus • Calcification may be tumours. present • Can haemorrhage • In children, 80% occur in Unlike ependymomas- trigone of lateral ventricle • th don’t extend into adjacent • In adults -4 ventricle more common in a 9 month old boy parenchyma, don’t seed thru CSF • Treatment – total surgical resection Lateral ventricle subependymoma in a 44- Smith et al Radiographics 2013 Smith et al 2013 year-old man with a history of headaches. Choroid Plexus Tumours Choroid Plexus Tumours

Intraventricular papillary neoplasms derived from choroid plexus epithelium • Papilloma (CPP) WHO grade 1 • Atypical papilloma WHO grade 2 • Carcinoma (CPCa) WHO grade 3

Cauliflower like fleshy lobulated intraventricular mass Can haemorrhage, necrosis, 64 yr/M with instability of gait x 2 years calcification CPP of 4th ventricle

Hydrocephalus common- overproduction of CSF, blockage due Choroid plexus papilloma in a 9 month old boy Enhancing intraventricular mass Hydrocephalus CT- to proteinaceous exudate, CSF seeding haemorrhage or direct obstruction 75% iso -hyperdense Calcification 20-25%

Smith et al Radiographics 2013 Cysts, necrosis, haemorrhage

Choroid Plexus Tumours Subependymal Giant Cell Tumour

• Intraventricular glioneuronal • Can have CSF seeding- tumour arising near foramen of image the entire Monro neuroaxis • 1.4% of all pediatric brain tumours • Incidence of SEGA- up to 15% of patients with Tuberous • Difficult to differentiate sclerosis CPP from CPCA on • Enlarging enhancing intraventricular mass in a imaging alone patient with Tuberous sclerosis (other findings of TS- cortical tubers, subependymal nodules) • Probably arises from a • Heterogeneity, brain subependymal nodule invasion, CSF seeding • WHO grade 1 tumour favour carcinoma CPCa in a 1 year old child • No CSF seeding Case courtesy Zoran Rumboldt Smith et al. Radiographics 2013

Subependymal Giant Cell Tumour Intraventricular meningioma Case B Case A • 0.5%– 4% of intracranial • Most common location - atrium of the lateral ventricles • Female : male = 2:1 • Peak age 30–60 years • Uncommon in pediatric age group but higher risk of sarcomatous change Location Well marginated, lobulated Consider Heterogeneous, strong enhancement type 2 Calcification Obstructive hydrocephalus Differentiated from hamartoma by interval slow growth- needs follow up Case courtesy Zoran Rumboldt Intraventricular meningioma “Central” Neurocytoma

• 0.25-0.5% of intracranial neoplasms • Lateral ventricles - may extend to 3rd ventricle- arise from septum pellucidum or lateral wall • Wide age range, mean 29 years Reduced diffusion Avid enhancement • Symptoms- from raised ICP Indolent, slow growing, large size Local or diffuse ventricular dilatation -obstruction of CSF flow • Usually gross surgical Cystic areas may be present resection is curative Periventricular edema- transependymal CSF flow or secretion Calcification in 50% of vascular endothelial growth factor by the meningioma CT iso- to hyperattenuating Smith et al Radiographics 2013

Central Neurocytoma Central Neurocytoma

• WHO grade 2 lesions • Well circumscribed, • Gross pathology- often lobulated mass friable with • Frequently cystic haemorrhage or changes- “bubbly” calcification appearance • Strong staining for • Calcification, synaptophysin is a haemorrhage diagnostic marker • Hyperattenuating on CT • Variable enhancement

Smith et al Radiographics 2013 30/F headaches. Central neurocytoma

Rosette-forming Glioneuronal Tumor Rosette-forming Glioneuronal Tumor

• Rare primary • Fairly well-circumscribed • WHO grade I • Arise from progenitor • Heterogeneous solid and pluripotential cells of the cystic mass subependymal plate • Glial and neuronal • Classically in the fourth differentiation, form small neurocytic rosettes ventricle • Young adults (mean age, 31.5 • Uncommon neoplasm, years) considered when a • Indolent cystic neoplasm of the • Present with signs of raised ICP or ataxia is • Location- fourth ventricle encountered in an adult • Gross total resection- curative

Smith et al Radiographics 2013 Smith et al Radiographics 2013 33 year old lady with recurrent GBM

Drop in 4th ventricle

62 year old lady

Tumours with CSF drop metastases Summary

• Ependymoma • Overlapping imaging features • Choroid plexus tumours • Age, Tumour location • Germinoma • • Pineoblastoma • Ependymal invasion- GBM

Recap Recap

• Infant (less than 1 year) with • Child (first 5 years) 4th tumour in lateral ventricles, ventricle plastic tumour- think hydrocephalus- think Ependymoma Choroid plexus tumour

Recap Recap

• In elderly, a poorly or not • In a patient with tuberous sclerosis, enhancing tumour in the foramen of Monro lesion think ventricle- think Subependymal Giant Cell Tumour Subependymoma (SEGA)

Recap Recap

• Tumour with homogeneous • Lateral ventricle tumour with enhancement , reduced diffusion- think bubbly appearance- think Meningioma Central neurocytoma

Take home message

• Diagnosis • Extension to parenchyma • Hydrocephalus, transependymal edema Thank you • Ependymal enhancement • CSF dissemination

Imaging of the whole neuroaxis