eEdE-85 John K. Fang, MD Wilson Altmeyer, MD Bundhit Tantiwongkosi, MD

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Presentation transcript:

Spectrum of Intraventricular Neoplasms: Narrowing the Differential Diagnosis eEdE-85 John K. Fang, MD Wilson Altmeyer, MD Bundhit Tantiwongkosi, MD Achint Singh, MD Carlos Bazan, MD

Disclosure Statement The authors have no financial interests to disclose.

Purpose To illustrate the spectrum of intraventricular neoplasms through discussion of their histologic origin and narrow the differential diagnosis through analysis of key imaging and demographic characteristics.

Approach and Discussion Ventricular histology Ventricular Wall and Septum Pellucidum Ependymoma Subependymoma Central Neurocytoma Subependymal Giant Cell Astrocytoma Choroid Plexus Choroid Plexus Papilloma Choroid Plexus Carcinoma Meningioma Metastasis Non-neoplastic intraventricular lesion Colloid Cyst

Histology Ependymal lining (ependymoma) Cuboidal/columnar cells variable in morphology according to location Lateral ventricles: ciliated and nonciliated ependymal cells, lack tight junctions Third ventricle: ventrolateral wall – tanycytes Extend from ventricular lining to underlying neurophils Provide neuroendocrine link with CSF

Histology Subependymal lining (subependymoma) – glial subependymal cells subjacent to ependymal lining Contributes to blood brain barrier Absent in portions of the third and fourth ventricles – no blood brain barrier Pineal gland, median eminence, subfornical organ, area postrema, subcommissural organ, organum vasculosum of the lamina terminalis, and posterior lobe of the pituitary gland

Histology Septum Pellucidum Shared origin with corpus callosum and limbic system Lined with ependyma Glial cells and neuronal precursor cells (central neurocytoma)

Histology Choroid Plexus Invaginations of pia mater about highly vascular stalks (metastasis) Continuous with ependymal lining Arachnoid cap cells (from arachnoid granulations) may be trapped during development (meningioma)

Ependymoma: Imaging Glial tumors arising from ependymal wall CT: Hypo to iso-attenuating Coarse calcifications typical (40-80%) May extend into parenchyma or through foramina

Ependymoma: Imaging T2: Iso-hyperintense with cystic components Occasional intratumoral hemorrhage Restricted diffusion: variable correlating with cellularity

Ependymoma: Imaging T1: Iso-hypointense Gd+: Heterogenous enhancement

Ependymoma: Categorization Supratentorial (40%) vs infratentorial (60%) Slightly less than 50% intraventricular Most common in children/young adults Infratentorial: 6 years Supratentorial: 18-24 years Clinical presentation: depending upon location Supratentorial: headache and seizure Fourth ventricle: hydrocephalus

Subependymoma: Imaging Glial tumors arising from subependymal layer CT: Iso-hypoattenuation Calcification possible but less common (30%) T1: Iso-hypointense Gd+: Most little to no enhancement

Subependymoma: Imaging T2: Hyperintense Typically no invasion of surrounding tissues No abnormal signal of periventricular tissues

Subependymoma: Categorization Most common in fourth ventricle (50-60%) and lateral ventricles (30-40%) Typically middle-aged males (2.3:1, M:F) Asymptomatic (60%) and incidental

Central Neurocytoma Arise from neuroprogenitor cells within septum pellucidum (“central”) CT: Hyperattenuating Calcifications (50%) Cystic changes common

Central Neurocytoma: Imaging T2: Hyperintense Bubbly cystic appearance

Central Neurocytoma: Imaging T1: Isointense Gd+: Moderate to strong enhancement

Central Neurocytoma: Categorization “Central” neurocytoma reserved for intraventricular origin, 50% lateral ventricle Similar tumors may occur elsewhere Mean patient age 29 (20-40) Clinical presentation: hydrocephalus and increased intracranial pressure

Subependymal Giant Cell Astrocytoma Subependymal Giant Cell Astrocytoma (or Tumor) Arise from neuroglial cells Found associated with tuberous sclerosis Numerous subcortical tubers

Subependymal Giant Cell Astrocytoma Arise near Foramen of Monro CT: Hypo-isoattenuation Calcification variable T1: Hypo-isointense T2: Iso-hyperintense Gd+: Avid enhancement

Subependymal Giant Cell Astrocytoma All occur in lateral ventricle near Foramen of Monro Associated with children with tuberous sclerosis

Choroid Plexus Papilloma: Imaging Arise from choroid plexus epithelium Typically centered in atria of lateral ventricles or fourth ventricle CT: Iso-hyperattenuation Calcification (24%)

Choroid Plexus Papilloma: Imaging T2: Iso-hyperintensity Papillary or lobular contour Flow voids common Often demonstrate extraventricular extension and edema Often associated with hydrocephalus Hydrocephalus

Choroid Plexus Papilloma: Imaging T1: Iso-hypointense Gd+: Avid enhancement

Choroid Plexus Papilloma: Categorization Lateral ventricle (50%) and fourth ventricle (40%) Lateral ventricle: 50% are < 10 years of age Fourth ventricle: Wide range (0-50 years) Clinical Presentation: Hydrocephalus and increased intracranial pressure

Choroid Plexus Carcinoma: Imaging Typically infants, young children Similar appearance to choroid plexus papillomas More invasive and irregular contour Difficult to differentiate from choroid plexus papilloma on imaging alone

Choroid Plexus Carcinoma: Imaging T2: Heterogenous attenuation Vasogenic edema and mass effect

Choroid Plexus Carcinoma: Imaging Gd+: Avid enhancement Can see invasion of brain parenchyma

Choroid Plexus Meningioma Arising from arachnoidal cap cells Trapped in choroid plexus during embryogenesis CT: Hyperattenuating Calcifications common (50%) Radiographics, Smith et al..

Choroid Plexus Meningioma T1: Iso-hypointense T2: Iso-hyperintnese Gd+: Avid enhancement May exhibit periventricular edema Varying degrees of ventricular dilation

Choroid Plexus Meningioma Most common in atria of lateral ventricles Less common third then fourth ventricles Female predominance (2:1, F:M) Adults: 30 - 60 years Clinical presentation: Increased intracranial pressure – usually large size at diagnosis

Intraventricular Metastasis Atrial mass in choroid plexus May be indistinguishable from choroid plexus tumors CT: Iso-hyperattenuation T1: Iso-hypointense T2: Variable - hyperintense Gd+: Avid enhancement Adults > children Renal and lung metastasis most common Known primary neoplasm raises concern for metastasis

Non-neoplastic Intraventricular Lesion Colloid Cyst Mucin containing cyst Third ventricle at foramen of Monro CT: hyperdense T1: hyperintense T2: variable Gd+: no enhancement Associated with hydrocephalus if obstructive

Summary Demographics CT MR and Gd+ Key Location and Characteristics Ependymoma 4th ventricle: 6 years Supratentorial: 18-24 Isoattenuation Calcifications 40-80% Heterogenous; intense enhancement 60% 4th ventricle Extraventricular extension common Subependymoma Middle aged males Iso-hypoattenuation Calcifications 30% Hydrocephalus 85% T1: hypo; T2: hyper Variable, usually little enhancement Majority in 4th and lateral ventricles Rarely extraventricular Central Neurocytoma 20-40 years Hyperattenuation Calcifications 50% T1: hyper; T2: heterogenous Moderate to strong enhancement 50% lateral ventricles and septum pellucidum SEGA Children with tuberous sclerosis Calcified nodule T1: hypo T2: heterogenously hyper Intense enhancement Lateral ventricle near foramen of Monro CP Papilloma Lateral: 50% < 10 years 4th: 0-50 years Iso-hyperattenuating Calcification 24% Hydrocephalus T1: Iso-hypo T2: variable hyper 50% lateral ventricles (atria) 40% 4th ventricle Prominent lobulations CP Carcinoma Infants/young children Heterogenous Vasogenic edema Aggressive, brain invasion Meningioma Adults 30-60 years Calcification 50% T2: Iso-hyper Atrial mass / choroid plexus Metastasis More common in adults Iso-hyperattenuation Primary lesions: lung / renal

References Koeller, Kelly and Glenn Sandberg. Cerebral Intraventricular Neoplasms: Radiologic-Pathologic Correlation. Radiographics. 22:1473-1505. 2002. Smith, Alice et al. Intraventricular Neoplasms: Radiologic-Pathologic Correlation. Radiographics. 33:21-43. 2013. Mortazavi, MM et al. The ventricular system of the brain: a comprehensive review of its history, anatomy, histology, embryology, and surgical considerations. Childs Nerv Syst. 30:19-35. 2014. Sarwar, Mohammad. The Septum Pellucidum: Normal and Abnormal. AJNR. 10:989-1005. 1989.