Brain+Tumors

=Goals: Approach=

6. Tissue Diagnosis in Treatment Planning
=Goals: Tumor Types=

3. Know difference between glial vs non-glial neoplasms, in terms of growth pattern, invasiveness, location, and prognosis.
I. Astrocytomas > From Astgrocytes > Fibrillar cellular Processes II. Glioblastoma > Most malignant astrocytoma > Necrosis > Mitoses > Vascular proliferation > Pleomorphic

III. Oligodendroglioma > Fried-Egg Cells > Chicken-wire Vasculature

IV. Ependymoma > Periveascular rosettes > True ependymal rosettes wtih lumans

V. Medulloblastoma > 'Blue-Cell' tumor > Fibrillar (Homer Wright) rosettes

VI. Primary Brain Lyhoma > BLue Tumor Discohesive Cells > B-cell markers

VII. Meningioma > Syncytial > Epethilial Whorls > Psamoma bodies VIII. Metastatic Carcinoma > Cohesive, non-invasive > Necrosis > Mitoses IX. Schwannoma > Verocay Bodies > Antoni A & B

Diffuse Fibrillary Astrocytomas Incidence: 40% of primary interacranial neoplasms 4 per 100000 person-years usually cerebral hemisphers with subcortical epicenter, but also neuraxis, includeing brain stem, cerebellum, spinal courd

Distribution M:F ratio 3:2 Any age, peaks in older pts Older pts -> higher grade

Clinical Presentation dependent on location Most common are foal neural deficits...motor, cranial nerve, sensory, visual, increased intracranial pressure, seizures Generalized neuorlogical signs, suptoms of increaed intracranial pressure include headaches, nausea, papilledema Acute onset suggests higher grade

Radiology Grade II tumors typtically nonenhancing masses with increased 52 waited, FLAIR on MRI Grade III nonenhancing or focally enhancing Glioblastoma multforme usually ring enhaning, sometimes 'butterfy lesion crossing corpus callosum Multifoal gliomas usually show unconnected foci if treated with radiation, radiation lesions ay appear similar to tumors

Prognosis: Survival inversely proportional to pt age Grae I:I : 5-10 y Grade II: 2-3 y Grade 1: < 1 y Pure astrocytomes have worse prognosis than ixed oligoastrocytomes, aoligodendrogliomas neurologic function indicates prognosis total resection may be beneficial radiotherapy often used also Grade III or IV more chemosensitiave

Pilocytic Astrocytoma 2% primary intracranial neoplasms 0.3 per 100000 person-years often cerebellar, optic pathway, hypothalamus/3rd ventrical, dorsal brain stem, spinal courd, sometimes cerebral hemisphere no gender association median age 13 years at diagnosis
 * Benign astorcyti neoplasm with hair-like processes, relatively circumscribed

clinical mansifestations based on location typcially insidious increased incidence in NF1 patients

Radiology Enhancing mural nodule w/ cyst \non-infiltrative appearance....well-demarcated "optic gliomas" often diffuse or fusiform enlargement of optic nerve, or chiasm

Prognosis 80$ 20 year survival rate most surgically curable raidation used for subtotally resected or recurrent cases no current chemotherapy

diffusely infiltartive glioma
 * Oligodendroglioma**

10% to 25% of diffuse gliomas 0.6 per 100,000 person-years crebreal hemisphers with prominent corticotropissm 1/2 in frontal lobes rare in brain stem, cerebellum, spinal courd 3:2 male: female peak onset 40 too 45 years, rare in children

Clinical manifestations based on location focal neural deficites, incraesed ICP, seizures > headaches, nausea, vomiting, papiledema rapid onset and progreession suggests anaplastic (Grade III) grade II typically nonenhancing masses with increased signal on T2, FLAIR MRI anaplastic (Grade III) typically enhancing Intratumoral calcifications common

Prognosis Surivval inversely proportional to age survival aerages 10-15 y for grade II, 3-5y for grade III oligodendrogliomas have better prognosis than astrocytomas better preoperative neurlogical performance incdicates better prognosis total resection my be beneficial radiothrapy used in pts w/ subttotal resection, or older pts chemotherapy often used, particularly those with 1p/19q chromosome deleitons (better prognosis)

Wll circumscribed malignant glioma with ependymal features
 * Ependymoma**

Incidence 3% to 9% of primary brain tumors, 5% to 12% in kids, 30% in infants most common glioma of spinal cord-50 to 60% of all cases 0.3 per 100,000 py often inn posterior fossa in kids, spinal cords in adults supratentorial examples often cystic, anaplastic

No sex predilaection peaks of onset at 0 to 16 and 30-40 y for intracranial and spinal ependymomas

Clinical Based off location Posterior fossa; cerebellar ataxia, hydrocephalus, increased ICP spinal: motor/senosry deficits

Radiological sometimes calcification hemorrhage
 * Enhancing, solid, well-demarcated mass
 * Supratentorial cases ofgten cystic
 * Spinal tumors are intra-axial sausage-shaped expansions of the cord, sometimes associated with adjacent syrinx

worse in kids, esp < 3 yo better in spinal than intracranial grading less predictve than other gliomas therapy primarily srgical, then loal irradiation craniospinal radiotherapy used if eidence of dissemination
 * Extent of resection is most poerful prognositc vbariable

Slow growing, dural tumors from arachnoid cap ells
 * Meningioma**

13 5o 26 % of primary intracranial tumors 6 per 100,000 py most arise proximal to dura within cranialm orbital, vertebral cavities common sites: parasaggital region, cavernous sinus, tuburculum sellae, lamina cribrosa, foramen magnum, torcular zone

F/m 3:2 any age most common in 6th, 7th decades atypcial, malignant more common in males

clinical based off location focal neural deficints, Incrased ICP, seizures May cause hyperostosis of overlying skull

Radiological Circumscribed isodense dural masses that enhance with contrast sometimes alcigication, bone, or cartilage dural tail-wedge shaped extension of tumor at edge, contrast ebgancing malignant and invasive tmors associated wtihc erebral edema

Most are curable by total resection good resection -> good prognosis rare variants...papillary, chordoid, rhabdoid, clear cell, atypical, and anaplastic may recur, metastacze: recurrence rate: grade I: 7 to 20%, grade II: 29 to 40 %, greade III: 50 to 78% higher cell prolifeative labelling correlates with incrased recurrence radiotherapy for higher grade, aggressive tumors

AKA neurilemoma,acoustic neuroma
 * Schwannoma**

most arise in association w/ peripheral neruves 8% intracranial, 29% spinal tumors association wit neurofibromatosis type II

no gender predilattion for spinal tumors intracranial tumors 2:1 f:M any age, peak between 40, 60 yo

commonly occur as asymptomatif masses sometimes pain, cord compression Nerve VIII tumors: hearing loss, tiniitus, facial paresthesias

Rdiology: well-circumscribed, heterogenously enhancitgn, sometimes cstic excellent prognosis usually surgically cureable

Increaseing incidence 0.8 to 5.5 % of primary intracranial neoplasms associated wtih AIDS, EBV-related tumors: 2% to 12% of AIDS tpts 10% to 12% of post-transplant lyphproliferative disorders confined to CNS 60% of primary CNS lympomas involve supratentoral space...ost often frontal lobe 25-50% multifocal 30% to 40% of primary CNS lympomas spread to leptomeninges
 * Primary CNS Lymphoma**

M/F 3:2 all ages affected, peak 60s-70s

clincal: focal neurlogical deficits minority have neurpsychiatric symptoms increased ICP, sizures possible 5-20% initially have eye involvement

Radiologica: solid or mutlipe hyperdense or isodense lesions with variable amounts of enhancement.

Prognosis: better if single leasion, no meningeal/periventricular involvement, immuncom-etent, less than 60 YO, preoperative Kernofsk score > 70 yo current therapy: radiation, chemotherapy 2y survival 40% to 70%

5Y SURVIVAL RATE 205% OT 45% OFTEN INITIALLY STEROID RESPONSIVE, WHICH MAY MAKE A DIAGNOSIS ON BIOPSY SAMPLES DIFFICULT AFTER INITIATION OF STEROIDS immunocompromised iniduals median survival < 1 yaear

Metastatic Cancer Most common CNS tumor about 25% of individuals with systemic cancer have CNS invovlemt 4.1 to 11.1 per 100.00 py 80% of brain metasases in arterial border zone of cerebrum; 15% in cerebellum majority of cases are multifocal, mutiple ascular distributions occaisionally leptomeningeal/dural absed

slight male predominance increased with age

clincial based of location often focal neural deicits, headaches, metnal distubrances

Radiologic: dicreete, isodense or hpodense masses with foal enhancement, surrounding edema 1/2 pts have mutliple leisuons

T1 wt -> hypodense, T2-weighted -> hyperentense, contrast enhancing

Good prognosis: younger age, fewer leisons higher karnofsky score als sensitivy to therapy, progression of primary neoplasm mean survival is 3 to 6 months solitary metastases may bea amenable to surgical excision