Tumor



GLIAL TUMORS

|Tumor |% |Who? |Location |Features |Prognosis |

|Diffuse Astrocytoma |20% of gliomas | |white matter of cerebral |Diffusely infiltrate w/o clear margins → rarely resectable. |Grade is based on: |

| | | |hemispheres | |degree of hypercellularity |

| | | | |Tend to become more anaplastic. |cellular atypia |

| | | | | |+/- endothelial changes |

| | | | |Lack a diagnostic histo pattern. |+/- necrosis |

| | | | | | |

| | | | |Pattern of growth may be regionally variable in same tumor. | |

|Brainstem gliomas | |children in 2nd decade |pons |subtype of diffuse astrocytoma. | |

| | | | | | |

| | | | |Usually infiltrate widely. | |

| | | | |May span full range of histo grades (including glioblastoma). | |

| | | | |Location precludes attempt to surgically excise. | |

| | | | |Pts. present w/: | |

| | | | |CN palsies | |

| | | | |long tract signs | |

| | | | |gait abnLs | |

| | | | |emesis | |

| | | | |cerebellar signs | |

| | | | |(due to location in CNS axis) | |

|Pilocytic Astrocytoma | |children and young adults|Midline: |Generally Circumscribed. |Optic Nerve gliomas are often a/w Von |

| | | |cerebellum |Almost always low grade. |Recklinghausen’s dz (NF 1) |

| | | |hypothalamus |Distinct histo pattern. | |

| | | |optic chiasm | | |

| | | |optic nerves |biphasic growth pattern: | |

| | | |(throughout neuraxis) |loose and densely cellular areas randomly intermingled | |

| | | | | | |

| | | | |Microcystic areas → contain variable amts of mucinous proteinaceous | |

| | | | |material | |

| | | | | | |

| | | | |Astrocytes are elongated, bipolar, form Rosenthal Fibers → opaque, | |

| | | | |homogeneous, eosinophilic, carrot shaped or beaded masses | |

| | | | |(ultrastructuarlly → dense arrays of greatly thickened, unusu. e dense | |

| | | | |glial filaments) | |

|Tumor |% |Who? |Location |Features |Prognosis |

|Cerebellar Pilocytic | |older children in 2nd | |Most common astrocytoma in childhood. |Good Px – even after only partial |

|Astrocytoma | |decade | |~60% are cystic |removal |

| | | | |Micro→ appear virtually identical to pilocytic astrocytomas in | |

| | | | |hypothalamus. | |

| | | | | | |

| | | | |Endothelial proliferation and | |

| | | | |modest cellular pleomorphism may be seen →does not necessarily indicate | |

| | | | |higher grade | |

|Subependymal Giant Cell |rare | | |Vascular, intraventricular mass. | |

|Astrocytoma | | | |Strong a/w Tuberous Sclerosis. | |

| | | | |Cell are very large | |

| | | | |strange mixture of astrocytic and neuronal characteristics | |

| | | | |Cytologically bizarre BUT benign in biologic behavior | |

| | | | |Serious clinical problems because of: | |

| | | | |location | |

| | | | |tendency to hemorrhage | |

|Glioblastoma Multiforme |65% of all primary |adults |supratentorial |Most common primary glioma. |Most invasive and aggressive of |

|(GBM) |glial tumors |peak incidence in 6th | |Rapid growth |gliomas. |

| | |decade | |High mitotic rate |Grade IV |

| | | | |Necrosis |May cross corpus collosum |

| | | | |Vascular endothelial proliferation | |

| | | | |Dense hypercellularity |heavily infiltrate the subpial zones |

| | | | |High degree of anaplasia |extend along perivascular |

| | | | |Hemorragic foci are common |(Virchow-Robin) spaces |

| | | | |May be regionally variable: |Invade leptomeninges and pachymeninges |

| | | | |zones of: |seed CSF |

| | | | |more primitive neuroectodermal tumor | |

| | | | |differentiated astrocytoma |Always recurs, even w/ apparent total |

| | | | |oligodendroglioma |surgical removal. |

| | | | |ependymoma | |

| | | | | |Survival: |

| | | | |More differentiated areas may dominate periphery → careful w/Dx in |w/o tx: 3 mos |

| | | | |superficial Bx |w/surg: 6 mos |

| | | | | |w/surg + radiation: 10-12 |

|Tumor |% |Who? |Location |Features |Prognosis |

|Oligodendro-glioma |5% of primary CNS |usu. adults |any location |Focal calcification → seen on plain film/CT scans | |

| |neoplasms |unusu in kids |m/c in cerebral white matter |Fried Egg appearance in “pure” well-differentiated →regular and round |Median post-op survival is 3-5 years |

| | |but any age | |nuclei | |

| | | | |→cytoplasm is water cleared – perinuclear halo |GOOD Px: |

| | | | |Highly vascularized |LOH1p |

| | | | |Slow growing |LOH19q |

| | | | |Some may be highly aggressive |respond well to chemo |

| | | | | |95% Five year survival |

| | | | |occur typically as grade II | |

| | | | |Anaplastic variants occur |BAD Px |

| | | | |No histo predicts behavior |CDKN2A deletion |

| | | | | |aggressive |

| | | | | |surival ................
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