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Spectrum of Intraventricular Neoplasms: Narrowing the Differential Diagnosis John K. Fang, MD Wilson Altmeyer, MD Bundhit Tantiwongkosi, MD Achint Singh, MD Carlos Bazan, MD eEdE-85 Home Disclosure Statement • The authors have no financial interests to disclose. Home 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. Home 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 Home 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 Home 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 Home Histology Septum Pellucidum • Shared origin with corpus callosum and limbic system • Lined with ependyma • Glial cells and neuronal precursor cells (central neurocytoma) Home 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) Home Ependymoma: Imaging • Glial tumors arising from ependymal wall • CT: Hypo to isoattenuating • Coarse calcifications typical (40-80%) • May extend into parenchyma or through foramina Home Ependymoma: Imaging • T2: Iso-hyperintense with cystic components • Occasional intratumoral hemorrhage • Restricted diffusion: variable correlating with cellularity Home Ependymoma: Imaging • T1: Iso-hypointense • Gd+: Heterogenous enhancement Home 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 Home 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 Home Subependymoma: Imaging • T2: Hyperintense • Typically no invasion of surrounding tissues – No abnormal signal of periventricular tissues Home 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 Home Central Neurocytoma • Arise from neuroprogenitor cells within septum pellucidum (“central”) • CT: Hyperattenuating • Calcifications (50%) • Cystic changes common Home Central Neurocytoma: Imaging • T2: Hyperintense • Bubbly cystic appearance Home Central Neurocytoma: Imaging • T1: Isointense • Gd+: Moderate to strong enhancement Home 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 Home Subependymal Giant Cell Astrocytoma • Subependymal Giant Cell Astrocytoma (or Tumor) – Arise from neuroglial cells • Found associated with tuberous sclerosis Numerous subcortical tubers Home Subependymal Giant Cell Astrocytoma • Arise near Foramen of Monro • CT: Hypo-isoattenuation – Calcification variable • T1: Hypo-isointense • T2: Iso-hyperintense • Gd+: Avid enhancement Home Subependymal Giant Cell Astrocytoma • All occur in lateral ventricle near Foramen of Monro • Associated with children with tuberous sclerosis Home Choroid Plexus Papilloma: Imaging • Arise from choroid plexus epithelium • Typically centered in atria of lateral ventricles or fourth ventricle • CT: Iso-hyperattenuation – Calcification (24%) Home 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 Home Choroid Plexus Papilloma: Imaging • T1: Iso-hypointense • Gd+: Avid enhancement Home 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 Home 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 Home Choroid Plexus Carcinoma: Imaging • T2: Heterogenous attenuation • Vasogenic edema and mass effect Home Choroid Plexus Carcinoma: Imaging • Gd+: Avid enhancement • Can see invasion of brain parenchyma Home Choroid Plexus Meningioma • Arising from arachnoidal cap cells – Trapped in choroid plexus during embryogenesis • CT: Hyperattenuating • Calcifications common (50%) Radiographics, Smith et al.. Home Choroid Plexus Meningioma • T1: Iso-hypointense • T2: Iso-hyperintnese • Gd+: Avid enhancement • May exhibit periventricular edema • Varying degrees of ventricular dilation Home 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 Home 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 Home 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 Home 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% T1: Iso-hypo T2: variable hyper Intense enhancement 50% lateral ventricles (atria) 40% 4th ventricle Extraventricular extension common Prominent lobulations Hydrocephalus CP Carcinoma Infants/young children Heterogenous Heterogenous Vasogenic edema Intense enhancement Aggressive, brain invasion Meningioma Adults 30-60 years Hyperattenuation Calcification 50% T1: Iso-hypo T2: Iso-hyper Intense enhancement Atrial mass / choroid plexus Metastasis More common in adults Iso-hyperattenuation T1: hypo; T2: hyper Intense enhancement Atrial mass / choroid plexus Primary lesions: lung / renal Home 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. Home
