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Glial and Glioneuronal Tumors

Glial and Glioneuronal Tumors. Neuropathology 4/18/2016 Megan Mantica , PGY3. Glial and Glioneuronal Tumors. Diffuse Fibrillary Astrocytoma Pilocytic Astrocytoma Subependymal Giant Cell Astrocytoma Pleomorphic Xanthoastrocytoma Oligodendroglioma Ependymoma Subependymoma

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Glial and Glioneuronal Tumors

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  1. Glial and Glioneuronal Tumors Neuropathology 4/18/2016 Megan Mantica, PGY3

  2. Glial and Glioneuronal Tumors • Diffuse Fibrillary Astrocytoma • Pilocytic Astrocytoma • Subependymal Giant Cell Astrocytoma • Pleomorphic Xanthoastrocytoma • Oligodendroglioma • Ependymoma • Subependymoma • Ganglioglioma

  3. Diffuse Fibrillary Astrocytoma • Diffuse infiltrative glioma displaying predominantly astrocytic features • Clinical Features: • Grade II-IV; 40% primary intracranial tumors • Incidence 4/100,000, median age 30-40 astrocytoma, 40-50 anaplastic astrocytoma, 50-60 GBM • Symptoms: new onset seizure, deficits based on location, ICP (HA, N/V, papilledema) • Imaging: • Grade II astrocytomas commonly non-enhancing, ill-defined, predominantly subcortical hemispheric masses • Anaplastic astrocytomas may show punctate or irregular foci contrast enhancement • GBM typically ring-enhancing, central low density necrosis, crosses corpus callosum (“butterfly lesions”) • Prognosis/Treatment: • Survival inversely proportional to patient age; Grade II 5-10Y survival, Grade II 2-3Y, Grade IV 1 Y • Astrocytomas worse prognosis than oligoastro and oligos • Treatment: gross total resection, RT + TMZ (for grade III, IV)

  4. A. Diffuse Astrocytoma, grade II, T2 ill-defined margins • B. GBM, T1 ring enhancing lesion, crosses corpus callosum • C. Radiation necrosis

  5. Diffuse Fibrillary Astrocytoma • Gross Findings: • Ill-defined borders, foci of tumor grossly invisible • Most common in WM or deep gray matter (thalamus, BG); blurring of corticomedullary and gray-white junction • Mass effect w/ MLS, blunting sulci, compression of ventricles • Variegated appearance w/ foci of necrosis and hemorrhage s/o GBM

  6. Diffuse Fibrillary Astrocytoma Microscopic Features Immunohistochemical GFAP: usually positive S-100 protein: positive; less specific, more sensitive than GFAP for glial Cytokeratin, EMA, HMB-45, melan A, LCA: negative Neurofilament highlights entrapped axons, c/w an infiltrative pattern p53 protein: positive in > 50% MIB-1 (Ki-67) labeling index roughly proportional to grade • Hypercellular, infiltrative • Hyperchromic oval-spindled nuclei w/ irregcountours • Nuclear atypia • Mitotic features, increased proliferation in high grade tumors • Endothelial hyperplasia/necrosis in GBM

  7. Diffuse Fibrillary Astrocytoma Genetics DDx Oligo/oligoastrocytoma Metastatic carcinoma or melanoma CNS lymphoma Radiation necrosis Reactive gliosis • Mutations p53 in 40-50% in astrocytomas, anaplastic astrocytomas and secondary GBM • EGFR amplification in 30-40% primary GBM • 10q deletion/LOH in majority of GBM • Loss of p16 in anaplastic astros and GBM

  8. GBM • Permanent • H&E • GFAP • Ki67 • P53 • Intraop • H&E

  9. Glial and Glioneuronal Tumors • Diffuse Fibrillary Astrocytoma • Pilocytic Astrocytoma • Subependymal Giant Cell Astrocytoma • Pleomorphic Xanthoastrocytoma • Oligodendroglioma • Ependymoma • Subependymoma • Ganglioglioma

  10. Pilocytic Astrocytoma • Benign astrocytic neoplasm w/ hair-like processes and circumscribed growth pattern • Clinical Features: • 2% primary intracranial neoplasms • Incidence 0.3/100,000 • Located in cerebellum, optic pathway, hypothalamus/3rd ventricle, tectum, spinal cord • Predominantly in children, median age 13Y • Associated with NF1 (optic pathway, brainstem) • Imaging: • Cyst w/ enhancing mural nodule • Well demarcated • Diffuse or fusiform enlargement of optic nerve/chiasm • Prognosis/Treatment: • 80% 20 year survival • Most surgically curable • RT for subtotal resected or recurrent tumors

  11. Pilocytic Astrocytoma Gross Findings Microscopic Findings Dense and loose appearance, sharply cicrumscribed Piloid astrocytic cells, long thin hair-like processes Round-oval nuclei Rosenthal fibers, PAS+ eosinophilic granular bodies characteristic Mitotic figures uncommon Infarct-like necrosis in 5-10% GFAP: positive S-100: positive • Cystic, well demarcated • Spongy appearance d/t microcysts or hypervascularity • Pilomyxoidastrocytomas are solid/gelatinous and may infiltrate parenchyma

  12. Pilocytic Astrocytoma Genetics DDx Diffuse astrocytoma Oligo/oligoastro Pleomorphic xanthoastrocytoma Ganglioglioma Monomorphouspilomyxoid astrocytoma DNET Reactive pilocytic gliosis • Reduced expression of neurofibromin (protein product of NF1 gene) assoc w/ NF1, not sporadic tumors • Gain of Chr 6 and 10 • Frequent BRAF gene fusions

  13. JPA • Permanent • H&E • GFAP • Ki67 • P53 • Intraop • H&E

  14. Glial and Glioneuronal Tumors • Diffuse Fibrillary Astrocytoma • Pilocytic Astrocytoma • Subependymal Giant Cell Astrocytoma • Pleomorphic Xanthoastrocytoma • Oligodendroglioma • Ependymoma • Subependymoma • Ganglioglioma

  15. Subependymal Giant Cell Astrocytoma • Benign or hamartomatous astrocytic and partially neuronal intraventricular tumor • Clinical Features: • < 1% primary intracranial tumors • Lateral or 3rd ventricles near foramen of Monro • Symptoms – obstructive hydrocephalus, ICP • Almost exclusively seen in Tuberous Sclerosis • Imaging: • Enhancing intraventricular mass, well demarcated • Often calcified • May be seen with other s/s of TS (tubers, candle gutterings, gray matter heterotopia) • Prognosis/Treatment: • Most surgically curable, excellent prognosis

  16. Subependymal Giant Cell Astrocytoma Gross Findings Microscopic Features Epithelioid, gemistocyte-like, spindled cells arranged in sweeping fascicles Dysmorphic cells with neuron-like nuclei and astrocyte-like cytoplasm Perivascular pseudorosettes Mitotic figures/necrosis uncommon GFAP: positive in subset of cells S-100: positive Synaptophysin, neurofilament, NeuN, chromogranin: positive in subset of cells • Solid, well demarcated, often calcified • Candle gutterings (resemble wax drippings) throughout ventricular surface

  17. Subependymal Giant Cell Astrocytoma Genetics DDx Gemistocytic astrocytoma Ependymoma Intraventricular meningioma Central neurocytoma Subependymoma • No significant alterations known

  18. SEGA • Permanent • H&E • GFAP • Ki67 • Intraop • H&E

  19. Glial and Glioneuronal Tumors • Diffuse Fibrillary Astrocytoma • Pilocytic Astrocytoma • Subependymal Giant Cell Astrocytoma • Pleomorphic Xanthoastrocytoma • Oligodendroglioma • Ependymoma • Subependymoma • Ganglioglioma

  20. Pleomorphic Xanthoastrocytoma • Variant of astrocytoma w/ reticulin deposition and increased pleomorphism • Clinical Features: • < 1% primary intracranial tumors • Temporal lobes • Children, young adults • Chronic seizure disorder • Imaging: • Cyst with enhancing mural nodule, but may be solid • Superficial cortex, may attach to overlying dura • Well demarcated, often calcified • Prognosis/Treatment: • Grade II good prognosis; 15-20% malignant transformation to Grade III and may die of disease • Many surgically curable; adjuvant therapy for subtotal resection, recurrent, or anaplastic tumors

  21. Pleomorphic Xanthoastrocytoma Gross Findings Microscopic Features Pleomorphic with mixed glial and mesenchymal-like features Spindled and atypical gemistocyte-like or epithelioid cells PAS-+ eosinophilic granular bodies Increased intercellular reticulin deposition Rosenthal fibers (periphery of the tumor ) Xanthomatous (clear and foamy) astrocytes in ¼ tumors Mitotic figures and necrosis are uncommon GFAP + in subset cells S-100 positive Ki-67 index usually low • Solid and rubbery; may have a cystic component • Demarcated , often calcified

  22. Pleomorphic Xanthoastrocytoma Genetics DDx GBM/gliosarcoma Malignant fibrous histiocytoma/pleomorphic sarcoma Ganglioglioma Pilocytic astrocytoma w/ increased atypia Meningioma • No significant alterations known

  23. Glial and Glioneuronal Tumors • Diffuse Fibrillary Astrocytoma • Pilocytic Astrocytoma • Subependymal Giant Cell Astrocytoma • Pleomorphic Xanthoastrocytoma • Oligodendroglioma • Ependymoma • Subependymoma • Ganglioglioma

  24. Oligodendroglioma • Diffusely infiltrative glioma w/ predmoninantlyoligodendroglioma features • Clinical Features: • 10-25% diffuse gliomas, 0.6/100,000, M:F 3:2 • Cerebral hemisphers, frontal lobe predominance • Median age 40-45Y • Focal neuro deficits, ICP, seizures • Imaging: • Non-enhancing masses (grade II), anaplastic oligos (grade III) typically enhancing • Intratumoral calcifications common • Prognosis/Treatment: • Grade II 10-15Y survival, grade III 3-5Y • Oligo better prognosis than astrocytomas • Gross total resection; RT for subtotal resection, anaplasia, older age • Chemo

  25. Oligodendroglioma Gross Findings Microscopic Features Hypercellular, uniformly round nuclei Clear perinuclear halos “fried egg” appearance Microcalcifications common Branching capillary network resembling “chicken-wire” GFAP: negative S-100: positive Cytokeratins, EMA, HMB-45: negative P53 protein negative • Ill-defined borders • Tumor center in cortex or corticomedullary junction; blurring of G-W junctions • MLS, blunting sulci, compression of venticles • Foci of necrosis/hemorrhage suggest anaplasia

  26. Oligodendroglioma Genetics DDx Astrocytoma/oligoastro Small cell glioblastoma DNET Pilocytic astrocytoma Clear cell ependymoma Cntralneurocytoma Extraventricularneurocytoma Metastatic clear cell carcinoma CNS lymphoma Clear cell meningioma • Co-deletion 1p/19q in 50-80% associated with increased survival and treatment responsiveness • Loss of p16 in anaplastic/recurrent oligo

  27. Glial and Glioneuronal Tumors • Diffuse Fibrillary Astrocytoma • Pilocytic Astrocytoma • Subependymal Giant Cell Astrocytoma • Pleomorphic Xanthoastrocytoma • Oligodendroglioma • Ependymoma • Subependymoma • Ganglioglioma

  28. Ependymoma • Well circumscribed malignant glioma with ependymal features • Clinical Features: • 3-9% primary brain tumors overall; 6-12% in children, 30% in infants • Most common glioma of spinal cord (50-60% all cases) • 0.3/100,000 • Posterior fossa (children), spinal cord (adults) • Peak age 0-16 and 30-40Y • Imaging: • Enhancing, solid, well-demarcated mass • Spinal tumors intra-axial sausage shape expansion of cord; associated with adjacent syrinx • May have calcifications/hemorrhage • Prognosis/Treatment: • Better prognosis: older age, spinal cord rather than intracranial, extent of resection • Surgical resection followed by RT

  29. Ependymoma Gross Findings Microscopic Features Sharp demarcation Perivascular pseduorosette Ependymal rosettes in 5-10% Round-oval nuclei, clear vacuoles in signet ring cells represent enlarged intracellular lumina EM: long zipper-like desmosomes/intercellular junctions GFAP: positive S-100: positive • Well circumscribed, soft, tan-red mass • Hemorrhage/necrosis may be present • Associated syrinx

  30. Ependymoma Genetics DDx Diffuse astrocytoma Astroblastoma Oligo PNET Choroid plexus papilloma Pilocytic astrocytoma • Aneuploidy seen in Grade I and II • NF2 with increased susceptibility for spinal ependymomas • 22q loss, 1q gain common

  31. Glial and Glioneuronal Tumors • Diffuse Fibrillary Astrocytoma • Pilocytic Astrocytoma • Subependymal Giant Cell Astrocytoma • Pleomorphic Xanthoastrocytoma • Oligodendroglioma • Ependymoma • Subependymoma • Ganglioglioma

  32. Subependymoma • Benign intraventricular glioma w/ clusters of ependymal-like nuclei embedded in dense fibrillary matrix • Clinical Features: • <1% primary brain tumors • 4th ventricle > lateral ventricles; rare spinal cord • Onset middle age-elderly • Asymptomatic, incidental detection, may have obstructive hydrocephhalus • Imaging: • Non-enhancing nodular intraventricular mass • Foci of calcification, hemorrhage or both • Spinal tumors intraaxial sausage shaped expansion of cord; associated syrinx • Prognosis/Treatment: • Excellent prognosis, benign • Mixed ependymoma-subependymomas have malignant potnetial and should be treated as ependymomas

  33. Subependymoma Gross Findings Microscopic Features Sharp demarcation Microcytic spaces, hyalinized vessels Round-oval nuclei in cellular clusters, dense fibrillary matrix Normal ependymal lining overlying tumor GFAP: positive S-100: positive Ki-76 low • Firm, well-circumscribed, nodular/lobulated tan-red mass • Hemorrhage/calcifications • Associated syrinx

  34. Subependymoma Genetics DDx Ependymoma Diffuse astrocytoma • No consistent alterations

  35. Glial and Glioneuronal Tumors • Diffuse Fibrillary Astrocytoma • Pilocytic Astrocytoma • Subependymal Giant Cell Astrocytoma • Pleomorphic Xanthoastrocytoma • Oligodendroglioma • Ependymoma • Subependymoma • Ganglioglioma

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