Download - Non neuroepithelial tumors
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NON-NEUROEPITHELIAL TUMORS
R.Rengarajan
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WHO classification
• Tumors of the Sellar Region • Hematopoietic tumors • Germ Cell Tumors • Tumors of the Meninges • Non-menigothelial tumors of the meninges • Tumors of Cranial and Spinal Nerves • Local Extensions from Regional Tumors • Metastatic tumours • Unclassified Tumors • Cysts and Tumor-like Lesions
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• Tumors of the Sellar Region – Pituitary adenoma – Pituitary carcinoma – Craniopharyngioma
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• Hematopoietic tumors – Primary malignant lymphomas – Plasmacytoma – Granulocytic sarcoma – Others
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• Germ Cell Tumors – Germinoma – Embryonal carcinoma – Yolk sac tumor (endodermal sinus tumor) – Choriocarcinoma – Teratoma – Mixed germ cell tumors
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• Tumors of the Meninges – Meningioma • variants: meningothelial, fibrous (fibroblastic),
transitional (mixed), psammomatous, angiomatous, microcystic, secretory, clear cell, chordoid, lymphoplasmacyte-rich, and metaplastic subtypes
– Atypical meningioma – Anaplastic (malignant) meningioma
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• Non-menigothelial tumors of the meninges – Benign Mesenchymal
• osteocartilaginous tumors • lipoma • fibrous histiocytoma • others
– Malignant Mesenchymal • chondrosarcoma • hemangiopericytoma • rhabdomyosarcoma • meningeal sarcomatosis • others
– Primary Melanocytic Lesions • diffuse melanosis • melanocytoma • maliganant melanoma
– variant meningeal melanomatosis
– Hemopoietic Neoplasms • malignant lymphoma • plasmactoma • granulocytic sarcoma
– Tumors of Uncertain Histogenesis - hemangioblastoma
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• Tumors of Cranial and Spinal Nerves – Schwannoma (neurinoma, neurilemoma)
• cellular, plexiform, and melanotic subtypes
– Neurofibroma • circumscribed (solitary) neurofibroma • plexiform neurofibroma
– Malignant peripheral nerve sheath tumor (Malignant schwannoma) • epithelioid • divergent mesenchymal or epithelial differentiation • melanotic
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• Local Extensions from Regional Tumors – Paraganglioma (chemodectoma) – Chordoma – Chondroma – Chondrosarcoma – Carcinoma
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Primary CNS lymphoma
• Malignant primary CNS neoplasm composed of B lymphocytes
• Enhancing lesion(s) within basal ganglia, periventricular WM
• 90% supratentorial
• Frontal and parietal lobes most common
• Deep gray nuclei commonly affected
• Lesions cluster around ventricles, GM-WM junction
• Often involve, cross corpus callosum
• Frequently abut, extend along ependymal surfaces
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• NECT• Hyperdense classically• May be isodense• +/- Hemorrhage, necrosis (immunocompromised)
• CECT• Common: Moderate, uniform (immunocompetent)• Less common: Ring (immunocompromised)• Rare: Nonenhancing (infiltrative, mimics white matter disease)
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• MR Findings
• T1WI
• Immunocompetent: Homogeneous isointense/hypointense to cortex• Immunocompromised: Isointense/hypointense to cortex
– May be heterogeneous from hemorrhage, necrosis
• T2WI
• Immunocompetent: Homogeneous isointense/hypointense to cortex– Hypointensity related to high nuclear to cytoplasmic ratio
• Immunocompromised: Isointense/hypointense to cortex– May be heterogeneous from hemorrhage, necrosis– Ca++ may rarely be seen, usually after therapy
• Mild surrounding edema is typical
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• FLAIR• Immunocompetent: Homogeneous isointense/hypointense to cortex• Immunocompromised: Isointense/hypointense• May be hyperintense• Mild surrounding edema is typical
• T2* GRE: May see blood products or calcium as areas of "blooming" (immunocompromised)
• DWI: Restricted diffusion, low ADC map reported
• T1 C+• Immunocompetent: Strong homogeneous enhancement• Immunocompromised: Peripheral enhancement with central necrosis or homogeneous enhancement• Nonenhancement extremely rare• Lymphomatous meningitis is typically related to systemic disease
• MRS• NAA decreased, Cho elevated• Lipid and lactate peaks reported
• MR perfusion: Early studies show increased rCBV
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Angiocentric lymphoma
• Rare malignancy characterized by intravascular proliferation of lymphoid cells with a predilection for CNS and skin
• A form of non-Hodgkin lymphoma (NHL) characterized by angiotropic growth
• Multifocal abnormal T2 hyperintensity in deep WM, cortex or basal ganglia + enhancement
• Supratentorial (periventricular/deep WM, G-W junction)
• May involve basal ganglia (BG), midbrain
• NECT: Focal, bilateral asymmetric low density lesions inWM, cortex, or basal ganglia
• CECT: Variable (none to moderate)
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• T1 WI• Multifocal hypointense lesions• May see blood products
• T2WI• 45% hyperintensities in deep WM (edema, gliosis)• 36% cortex hyperintensity, infarct-like lesions• May see hemorrhagic transformation
• T2* GRE: May see blood products "blooming“
• DWI: Diffusion restriction reported
• T1 C+• Variable enhancement: Linear, punctate, patchy, nodular, ring-like, gyriform, homogeneous• o Meningeal and/or dural enhancement
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Germinoma• Morphologic homologues of germinal neoplasms arising in the gonads and
extragonadal sites
• Pineal region mass that "engulfs" the pineal gland
• Midline near the 3rd ventricle - 80-90% (Pineal region - 50-65%, Suprasellar - 25-35%, Basal ganglia and thalami - 5-10%)
NECT• Sharply circumscribed dense mass (hyperdense to GM)• Pineal: Mass drapes around posterior 3rd ventricle or "engulfs" pineal gland• Suprasellar: Retrochiasmatic, non-cystic, non -calcified• ± Hydrocephalus
CECT• Strong uniform enhancement, ± CSF seeding• Cystic/necrotic/hemorrhagic components not uncommon with larger germinomas
(especially in basal ganglia)
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• T1Wl• Isointense or hyperintense to GM• Early cases may only show absent posterior pituitary bright spot
• T2Wl• Iso-to-hyperintense to GM (high nuclear:Cytoplasmic ratio)• Cystic or necrotic foci (high T2 signal)• Less common: Hypointense foci (hemorrhage)
• FLAIR: Slightly hyperintense to GM
• T2* GRE: Calcification, hemorrhage (rare)
• DWI: Restricted diffusion due to high cellularity
• T1 C+: Strong, homogeneous enhancement, ± CSF seeding, ± brain invasion
• MRS: inc Choline, dec NAA, ± lactate
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Teratoma
• Tridermal mass originating from displaced embryonic tissue that is misenfolded
• Midline mass containing: Ca++, soft tissue, cysts, and fat
• Hugs midline, optic chiasm, pineal gland (Majority are supratentorial)
• NECT: Fat, soft tissue, Ca++, cystic attenuation
• CECT: Soft tissue components enhance
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MR Findings
• T1WI: inc signal from fat, variable signal from Ca++
• T2WI: Soft tissue components iso- to hyperintense
• FLAIR: dec signal from cysts, inc signal from solid tissue
• T2* GRE: dec signal from Ca++
• T1 C+: Soft tissue enhancement
• MRS: inc lipid moieties on short echo
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Embryonal carcinoma
• Malignant tumor composed of undifferentiated cells
• Heterogeneous pineal or suprasellar mass in adolescent
• Hugs midline as other CNS GCTs
• Typically well circumscribed or lobulated
• NECT – Heterogenous - Isoattenuating to hyperattenuating
• CECT - Enhancing, ± cysts, hemorrhage
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T1WI• Hypointense to isointense to GM• T1 shortening due to protein, blood or fat
T2WI: Isointense to slightly hyperintense to GM
FLAIR• Hyperintense solid elements• ± Hydrocephalus
T2* GRE: Dephasing from hemorrhagic foci
DWI: ± Restriction within solid components
T1 C+: Heterogeneous enhancement, ± CSF spread
MRS: inc Choline, inc lipid and lactate, dec NAA
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Meningioma
• WHO grade 1 Meningioma
• Dural-based enhancing mass w/cortical buckling & trapped CSF clefts/cortical vessels
• Supratentorial (90%): Para sagittal/convexity (45%), sphenoid ridge (15-20%), olfactory groove (5-10%), parasellar (5-10%)
• Infratentorial (8-10%): CPA most common
• Misc inside the dural: Intraventricular, optic nerve sheath, pineal region
• Misc outside the dura: Paranasal sinus (most common), nasal cavity, parotid, skin, calvarium
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• NECT• Hyperostosis, irregular cortex, tumoral calcifications, inc vascular markings• Sharply circumscribed smooth mass abutting dura• Hyperdense (70-75%), iso- (25%), hypo- (1-5%)• Calcified (20-25%): Diffuse, focal, sandlike, sunburst, globular, rim• Necrosis, cysts, hemorrhage (8-23%)• Rare lipoblastic subtype • Brain cysts & trapped pools of CSF common• Peritumoral hypodense vasogenic edema (60%)
• CECT: > 90% enhance homogeneously & intensely
• CTA: May complement DSA in defining vascular supply to tumor & normal tissues from each feeder artery before embolization
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• T1WI• Usually iso- to slightly hypointense with cortex• Necrosis, cysts, hemorrhage (8-23%)• Best to visualize gray matter "buckling“
• T2WI• Variable; sunburst pattern may be evident• Necrosis, cysts, hemorrhage (8-23%)• Best to visualize trapped hyperintense CSF clefts (80%) & vascular flow voids
(80%)
• FLAIR: Hyperintense peritumoral edema, dural "tail“
• T2* GRE: Best sensitivity for calcification
• DWI: DWI, ADC maps for CM variable in appearance
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• T1 C+• > 95% enhance homogeneously & intensely• Dural "tail" (35-80% of cases ): Non-specific• En plaque: Sessile thickened enhancing dura
• MRV: Evaluate possible sinus involvement
• MRS• Elevated levels of Alanine at short TE
• Reported peak ranges from 1.3-1.5 ppm
• Perfusion MRI: Good correlation between volume transfer constant (K-trans) & histologic grade
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Atypical and malignant meningioma
Common meningioma = WHO grade 1 meningiomaAtypical meningioma = WHO grade 2 meningiomaMalignant meningioma = WHO grade 3 meningioma
• Dural based lesion locally invasive with areas of necrosis & marked brain edema
• Occur anywhere along neuraxis
• AM: Para sagittal (44%), cerebral convexities (16%)
• NECT• Hyperdense w/minimal or no calcification• Marked perifocal edema & bone destruction• CT "Triad" of MM: Extracranial mass, osteolysis, & intracranial tumor
• CECT• Enhancing tumor mass• Prominent pannus or tumor, extending away from mass termed "mushrooming"
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• T1WI• Indistinct tumor margins• Extending tumor interdigitating with brain
• FLAIR:Marked peritumoral edema
• DWI• Markedly hyperintense on DWI• Marked decrease in ADC• Correlates with histopathology
• T1 C+• Enhancing tumor mass• Plaque like & may extend into brain, skull, scalp
• MRV: Evaluate possible sinus involvement
• MRS - Elevated levels of Alanine at short TE• Reported peak ranges from 1.3-1.5 ppm
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Hemangioblastoma
• Vascular neoplasm of uncertain histogenesis
• Adult with intra-axial posterior fossa mass with cyst, enhancing mural nodule abutting the pia
• 90-95% posterior fossa ( 80% cerebellar hemisphere )
• 60% cyst with mural nodule ( 40% solid )
• NECT – low density cyst with isodense mural nodule
• CECT – Nodule enhances intensely, Cyst wall doesn’t enhance
• CTA – may demostrate arterial feeders
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T1 WI• Nodule isointense with brain• Cyst moderately hyperintense to CSF
T2 WI• Both nodule and cyst are hyperintense• Prominent flow voids in some cases
FLAIR• Both cyst and nodule hyperintense
T1 C+• Nodule enhances intensely• Solid enhancement pattern less common• Cyst wall enhancement very less common
• 20-40 % HGBL occur in VHL patients (multiple tumors)• With visceral cysts, RCC
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Hemangiopericytoma
• Sarcoma related to neoplastic transformation of pericytes
• Lobular enhancing extra-axial mass with dural attachment +/- skull erosion
• Supratentorial – occipital region most common
• NECT – hyperdense extra-axial mass with surrounding edema, calvarial erosion
• CECT – strong heterogenous enhancement
No Ca++ or hyperostosis
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• T1 WI• Heterogenous mass, isointense to gray matter
• T2 WI• Heterogenous isointense mass• Prominent flow voids are common• Surrounding edema, mass effect are common• Hydrocephalus
• T1 C+• Marked heterogenous enhancement• Dural tail seen in 50%
• MRV – occlusion of venous sinuses
• MRS – elevated myoinositol
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• Local recurrence common, 50-80%
• Extracranial metastases common, up to 30%• Commonly liver, lungs, lymph nodes, bones
Staging, Grading or Classification Criteria• WHO grade II or III (anaplastic)
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Thank you