Intraventricular tumours Kumar... · Intraventricular glioneuronal tumour arising near foramen of...
Transcript of Intraventricular tumours Kumar... · Intraventricular glioneuronal tumour arising near foramen of...
Intraventricular tumours Sumeet Kumar
National Neuroscience Institute
Duke-NUS Medical School
Singapore
Intraventricular tumours
• Ependymoma
• Subependymoma
• Choroid plexus Papilloma/Ca
• Subependymal Giant cell Tumour/ Astrocytoma (SEGA)
• Meningioma
• Central Neurocytoma
• Others- GBM, Lymphoma, RGNT (Rosette forming Glioneuronal Tumour), Metastases
Cells of origin of intraventricular tumours
• Epithelial lining of ventricles- ependyma → ependymoma
• Adjacent to ependymal lining- subependymal plate of glial cells → subependymoma
• Choroid plexus → choroid plexus tumours- papilloma, atypical papilloma, carcinoma
• Vascularity of choroid plexus → metastases
• Arachnoidal cap cells which form arachnoid granulations can be trapped in the choroid plexus → meningioma
• Septum pellucidum lined by glial cells and neuronal precursor cells → central neurocytoma
Ependymoma
• Arise from epithelial lining of ventricles- ependyma
• Parenchymal ependymomas arise from embryologically trapped ependymal cells
Ependymal cells lining the ventricles
http://missinglink.ucsf.edu/lm/introductionneuropathology
Ependymoma
• 3-5% of intracranial neoplasms
• 60% posterior fossa
• 40% supratentorial
• Half parenchymal and half intraventricular
• More common in children (1-6 years)
In the posterior fossa
• Supratentorial lesions- mean age 18-24 years
• Children have a worse prognosis- higher prevalence of anaplastic form
Ependymoma
• Gross pathology- soft “plastic” tumours, “toothpaste”tumours
• 4th ventricular tumours extend through foramen of Luschka into CP Angle or through foramen magnum
• Histology- perivascular rosettes and true ependymal rosettes
Osborn. Copyright Amirsys Inc 2004 http://missinglink.ucsf.edu/lm/introductionneuropathology
Ependymoma
Fill 4th ventricle like plaster cast
Extend thru the foramen of Luschka and
foramen magnum- NOT PATHOGNOMIC-
occasionally medulloblastomas also
Cystic components
frequent
Calcification
Hemorrhage
Mildly reduced
diffusion
Intraventricular
ependymomas can extend
into adjacent parenchyma
47/F 4th ventricular ependymoma with hydrocephalus
Ependymoma
Fill 4th ventricle like plaster cast
Extend thru the foramen of Luschka and
foramen magnum- NOT PATHOGNOMIC-
occasionally medulloblastomas also
47/F 4th ventricular ependymoma with hydrocephalus
Can seed through CSF (12%)
Imaging of entire neuroaxis
Subependymoma
• 0.2- 0.7 % of intracranial neoplasms
• Mostly incidental
• 50-60% in 4th ventricle
• 30-40% in lateral ventricles
• M:F= 2.3:1
• Most patients in 5th and 6th decades
• Prognosis- good
Subependymoma
• WHO grade 1 neoplasm
• Ependymal differentiation
• Well circumscribed, attached to the ventricle wall by a narrow pedicle
Osborn. Copyright Amirsys Inc 2004
Subependymoma
• Well circumscribed tumours
• Cystic change common
• Mostly no/ minimal enhancement
• Calcification may be present
• Can haemorrhage
• Unlike ependymomas- don’t extend into adjacent parenchyma, don’t seed thru CSF
Lateral ventricle subependymoma in a 44-
year-old man with a history of headaches. Smith et al 2013
Choroid Plexus Tumours
• 2-4% of pediatric brain tumours
• Most commonly child <5 years of age
• 20% of brain tumours occurring in the first year of life are Choroid plexus tumours.
• In children, 80% occur in trigone of lateral ventricle
• In adults -4th ventricle more common
• Treatment – total surgical resection
Smith et al Radiographics 2013
Choroid plexus papilloma in a 9 month old boy
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, calcification
Hydrocephalus common- overproduction of CSF, blockage due to proteinaceous exudate, haemorrhage or direct obstruction
Smith et al Radiographics 2013
Choroid plexus papilloma in a 9 month old boy
Choroid Plexus Tumours
Enhancing intraventricular mass
Calcification 20-25%
Cysts, necrosis, haemorrhage
Hydrocephalus
CSF seeding CT-
75% iso -hyperdense
64 yr/M with instability of gait x 2 years
CPP of 4th ventricle
Choroid Plexus Tumours
• Can have CSF seeding- image the entire neuroaxis
• Difficult to differentiate CPP from CPCA on imaging alone
• Heterogeneity, brain invasion, CSF seeding favour carcinoma
Case courtesy Zoran Rumboldt
CPCa in a 1 year old child
Subependymal Giant Cell Tumour
• Intraventricular glioneuronal tumour arising near foramen of Monro
• 1.4% of all pediatric brain tumours
• Incidence of SEGA- up to 15% of patients with Tuberous sclerosis
• Enlarging enhancing intraventricular mass in a patient with Tuberous sclerosis (other findings of TS- cortical tubers, subependymal nodules)
• Probably arises from a subependymal nodule
• WHO grade 1 tumour
• No CSF seeding
Smith et al. Radiographics 2013
Subependymal Giant Cell Tumour
Case courtesy Zoran Rumboldt
Case A
Case B
Differentiated from hamartoma by interval slow
growth- needs follow up
Location Well marginated, lobulated
Heterogeneous, strong enhancement
Calcification
Obstructive hydrocephalus
Intraventricular meningioma
• 0.5%– 4% of intracranial meningiomas
• 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 Consider neurofibromatosis type 2
Intraventricular meningioma
Indolent, slow growing, large size
CT iso- to hyperattenuating
Reduced diffusion
Calcification in 50%
Cystic areas may be present
Avid enhancement
Local or diffuse ventricular dilatation -obstruction of CSF flow
Periventricular edema- transependymal CSF flow or secretion
of vascular endothelial growth factor by the 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
• Symptoms- from raised ICP
• Usually gross surgical resection is curative
Smith et al Radiographics 2013
Central Neurocytoma
• WHO grade 2 lesions
• Gross pathology- often friable with haemorrhage or calcification
• Strong staining for synaptophysin is a diagnostic marker
Smith et al Radiographics 2013
Central Neurocytoma
• Well circumscribed, lobulated mass
• Frequently cystic changes- “bubbly” appearance
• Calcification, haemorrhage
• Hyperattenuating on CT
• Variable enhancement
30/F headaches. Central neurocytoma
Rosette-forming Glioneuronal Tumor
• Rare primary brain tumor
• WHO grade I
• Arise from progenitor pluripotential cells of the subependymal plate
• Glial and neuronal differentiation, form small neurocytic rosettes
• Young adults (mean age, 31.5 years)
• Indolent
• Present with signs of raised ICP or ataxia
• Location- fourth ventricle
• Gross total resection- curative
Smith et al Radiographics 2013
Rosette-forming Glioneuronal Tumor
• Fairly well-circumscribed
• Heterogeneous solid and cystic mass
• Classically in the fourth ventricle
• Uncommon neoplasm, considered when a cystic neoplasm of the fourth ventricle is encountered in an adult
Smith et al Radiographics 2013
62 year old lady Glioblastoma
33 year old lady with recurrent GBM
Drop metastasis in 4th ventricle
Tumours with CSF drop metastases
• Ependymoma
• Choroid plexus tumours
• Germinoma
• Medulloblastoma
• Pineoblastoma
• Ependymal invasion- GBM
Summary
• Overlapping imaging features
• Age, Tumour location
Recap
• Infant (less than 1 year) with tumour in lateral ventricles, hydrocephalus- think
Choroid plexus tumour
Recap
• Child (first 5 years) 4th ventricle plastic tumour- think
Ependymoma
Recap
• In a patient with tuberous sclerosis, foramen of Monro lesion think
Subependymal Giant Cell Tumour (SEGA)
Recap
• In elderly, a poorly or not enhancing tumour in the ventricle- think
Subependymoma
Recap
• Tumour with homogeneous enhancement , reduced diffusion- think
Meningioma
Recap
• Lateral ventricle tumour with bubbly appearance- think
Central neurocytoma
Take home message
• Diagnosis
• Extension to parenchyma
• Hydrocephalus, transependymal edema
• Ependymal enhancement
• CSF dissemination
Imaging of the whole neuroaxis
Thank you