Non neuroepithelial tumors

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Non neuroepithelial tumors

  1. 1. NON-NEUROEPITHELIAL TUMORS R.Rengarajan
  2. 2. 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
  3. 3. • Tumors of the Sellar Region – Pituitary adenoma – Pituitary carcinoma – Craniopharyngioma
  4. 4. • Hematopoietic tumors – Primary malignant lymphomas – Plasmacytoma – Granulocytic sarcoma – Others
  5. 5. • Germ Cell Tumors – Germinoma – Embryonal carcinoma – Yolk sac tumor (endodermal sinus tumor) – Choriocarcinoma – Teratoma – Mixed germ cell tumors
  6. 6. • Tumors of the Meninges – Meningioma • variants: meningothelial, fibrous (fibroblastic), transitional (mixed), psammomatous, angiomatous, microcystic, sec retory, clear cell, chordoid, lymphoplasmacyte-rich, and metaplastic subtypes – Atypical meningioma – Anaplastic (malignant) meningioma
  7. 7. • 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
  8. 8. • 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
  9. 9. • Local Extensions from Regional Tumors – Paraganglioma (chemodectoma) – Chordoma – Chondroma – Chondrosarcoma – Carcinoma
  10. 10. 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
  11. 11. • • • • 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)
  12. 12. • 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
  13. 13. • • • • • 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
  14. 14. 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)
  15. 15. • • • 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
  16. 16. 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 - 2535%, 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)
  17. 17. • 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
  18. 18. 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
  19. 19. 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
  20. 20. 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
  21. 21. 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
  22. 22. 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 (1520%), 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
  23. 23. • • • • • • • • • 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
  24. 24. • • • • 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
  25. 25. • • • • 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
  26. 26. Atypical and malignant meningioma Common meningioma = WHO grade 1 meningioma Atypical meningioma = WHO grade 2 meningioma Malignant 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"
  27. 27. • • • 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
  28. 28. 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
  29. 29. 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
  30. 30. 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
  31. 31. • 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
  32. 32. • 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)
  33. 33. Thank you

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