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Brain neoplasm

Supischa Theerasasawat

Outline
Epidemiology Classification Primary brain tumor and metastasis : nature , histological and radiological findings Treatment

Epidemiology
Central Brain Tumor Registry of the United States (CBTRUS), the annual rate was 11.47 per 100,000 persons Annual incidence (1995-1999) - Malignant brain cancer : 6.8 per 100,000 person-years - Benign brain tumor : 4.2 per 100,000 personyears

Epidemiology
Glioma : most common brain tumor astrocyte (astrocytoma), oligodendrocytes (oligodendroglioma), or ependymal cells (ependymoma) Astrocytomas : 80% of all malignant brain tumors

Types of intracranial tumor in the combined series of Zuch, Cushing and Olivecronav

Age-specific frequency of tumor types with age

The 2007 Revised World Health Organization (WHO) Classification of Tumours of the Central Nervous System: Newly Codified Entities

Mortality and prognostic factors


Brain tumors : 2% of all cancers The 5-year survival rates : 22-32% Favorable prognostic factors - young age - high Karnofky performance status - lower pathological grade - long duration of symptoms
- absence of mental changes - cerebellar location - small preoperative tumor - completeness of surgical resection

Mortality and prognostic factors


The cause of brain tumors : unknown Two clear causal factors : Ionizing radiation and hereditary predisposition Ionizing radiation : gliomas, meningiomas and schwannomas Hereditary predisposition (neurocutaneous syndrome) - NF1-2, TS, vHL, Li-Fraumenis syndrome, Gorlins syndrome

Primary brain tumor

Clinical features
Headache : referred frontal headache , cervical pain (CN IX and X) Seizure ~35%, focal and/or 2 GTC Cognitive dysfunction Nausea and vomiting Related to endocrine dysfunction Visual symptoms plateau wave : transient altered consciousness and visual disturbances

Tumor-related complications
Seizure Cerebral edema Venous thrombosis (DVT)

Investigation
CT or MRI EEG Evaluation of primary tumor (metastasis) Lumbar puncture

Tumors of neuroepithelial tissue


Arises from primary cells of the nervous system : glia, neuron or precursor cells Diffuse glioma (neuro-oncology) - diffuse astrocytoma - oligodendroglioma - oligoastrocytoma - all infiltrative gliomas

Diffuse astrocytoma (WHO grade II)


Slow growing tumor Regularly undergoes malignant progression Age of onset : 35 y/o Survival : 5-10 years Diffuse : poorly circumscribed , diffusely infiltrative and lack of solid formation Gliomatosis cerebri

Diffuse astrocytoma (WHO grade II)


Radiographic finding - ill-defined T2W hyperintensity, non-enhancing cerebral mass with subcortical epicenter - minimal vasogenic edema Morphological type - fibrillary, gemistocytic, and protoplamic TP53 gene

Anaplastic astrocytoma (WHO grade III)


Similar to grade II astrocytoma Age of onset : decade later Survival : 3 years Contrast enhancement , more mass effect TP53 mutation, deletion of CDKN2A/p16 gene

GBM (WHO grade IV)


Most common in adult , 50% of all gliomas Age of onset : 50-60 y/o Survival : 1 year after treatment Supratentorial structure : deep white matter, basal ganglia, thalamus Multiforme : heterogeneous Gross : well-circumscribed, microscopically infiltrative Butterfly lesion : spread via corpus callosum to opposite site

GBM
Neuraxis seeding, meninges Heterogeneous signal on T1W, T2W and irregular ring enhancement (necrosis)+ mass effect and vasogenic edema Variants : giant cell glioblastoma, small cell glioblastoma, gliosarcoma (temporal) Genetically best characterized brain tumor Less TP53 mutation EGFR amplification, PTEN mutation

Oligodendroglioma (WHO grade II or III)


Most : high grade at time of diagnosis Young to middle-aged adult Hemispheric mass : frontal lobe Survival > 10 years Histological finding : uniform fried egg appearance, rich capillary network Calcification is common Loss of chromosome arms 1p and 19q

Ependymoma (WHO grade II or III)


4% of all brain tumors 3rd common in children (1st decade) Infratentorial site : most common in 4th ventricle Ependymal rosette Complication : hydrocephalus Seeding subarachnoid space Surgically curable Associated NF2 esp. spinal ependymomas

Neuronal/Glioneuronal tumors

Dysembryoplastic neuroepithelial tumor (WHO grade I)


DNT Benign quasihamartomatous tumor Childhood and early adult Excellent prognosis Supratentorial, intracortical : Temporal lobe long history of Intractable seizure Multinodular, adjacent cortical dysplasia Biopsy : resemble oligodendroglioma Floating neuron, mucin rich cortical nodule No cortical/subcortical invasion

Embryonal tumors/Primitive Neuroectodermal tumors


PNET : umbellar term

Small blue cell tumors - Medulloblastoma (prototype) - peripheral nervous system PNET - CNS variants : supratentorial PNET (sPNET) : pineoblastoma, central neuroblastoma,
ependymoblastoma, medulloepithiloma - PNETs of special sensory organ : retinoblastoma, olfactory neuroblastoma - AT/RT

Medulloblastoma (WHO grade IV)


Most common form of PNET Age of onset :<10 y/o (18-25) By definition, originates in cerebellum Disseminated along CSF pathway 5-year survival : 70-80% Associated with form of Turcots syndrome, Gorlins syndrome MYCC and MYCN gene amplification : aggressiveness of medulloblastoma

Meningeal/extra-axial tumors
Most common : meningiomas Hemangiopericytoma, sarcoma, lymphoma, schwannoma

Meningioma (WHO grade I)


20-25% of all intracranial tumors age > 50 y/o , 80% benign 5-year recurrence rates 5% for gross totally resected, 30% subtotally resected Location : descending order of frequency - cerebral convexity , parasagittal region, sphenoid wing, parasellar region, and spinal canal Children : posterior fossa and lateral ventricle

Meningioma (WHO grade I)


Multiple meningioma : NF2 Meningioma en-plaque : diffuse, carpetlike tumor spread along dural surface WHO classification : 13 morphological types Histology : Psammoma body Losses of chromosome 22, tumor suppressor gene (NF2), DAL1 or protein 4.1B on 18p11.3 MRI : isointense T1W, T2W, homogeneous enhancement, dural based mass

Atypical meningioma (WHO grade II)


Increase risk of recurrence 15-20% of all meningiomas Mitotic index > 4 mitosis per 10 HPFs Deletion of 1q, 6p, 10 and 14q

Anaplastic meningioma (WHO grade III) : mitotic index > 20 per 10 HPFs, highly aggressive and infiltrative

Nerve sheath tumors


Schwannoma (NF2) Neurofibroma (NF1)

Schwannoma (neurilemoma) (WHO grade I)


Peak 40-50 y/o Vestibular portion of 8th cranial nerve Bilateral : NF2 Spinal schwannoma : dorsal root (sensory) Dumbell-shaped mass Pure schwann cell proliferation (opposed to neurofibroma)

Typically, capsule and push parent nerve aside

Neurofibroma (WHO grade I)


Pathognomonic of NF1 : Multiple spinal root involvement Fusiform intraneural mass Plexiform growth pattern, mixture of cell types (schwann cell, fibroblast, perineurial-like cell, mast cell) Entrap parent nerve

Miscellaneous tumors

CNS lymphoma
Primary lymphoma : multifocal, deep parenchyma, perivantricular, spare leptomeninges Systemic (2nd) lymphoma : leptomeninges, spare parenchyma Common site : epidural spine Immunocompromise : associated with EBV Diffuse large B cell lymphoma MRI : hypointense T2W, strong homogeneous enhancement, vasogenic edema

Germ cell tumors


School-aged children, boy Most common : pineal region, suprasellar/hypothalamic region Subarachnoid seeding Marker - germinoma : placenta alkaline phosphatase (PLAP) - yolk sac tumor : AFP - choriocarcinoma or syncytiotrophoblast : -HCG

Germ cell tumors


Germinoma :100% curable, radiosensitive Mixed germ cell tumors , mature teratoma

Hemangioblastoma (WHO grade I)


Peak 40 y/o VHL disease Benign, highly vascular tumor Most common : cerebellum, spinal cord, retina Cystic tumor with enhancing mural nodule VHL gene on 3p25-26 (tumor suppressor) Renal cell carcinoma, pheochromocytoma

Craniopharyngioma (WHO grade I)


Any age (1st two decade) Remnant of Rathkes pouch Location : suprasellar, intrasellar, optic chiasm Complication : hydrocephalus (3rd ventricle) Calcified, cystic, solid component

Management
Surgery : primary modality Biopsy : stereotactic , <2% seizure, ICH, infection Resection : complete resection - ganglioglioma, choroid plexus papilloma, PXA, pilocytic astrocytoma, DNT, pituitary adenoma, meningioma - subtotal resection : infiltrative increase ICP. ICH

Management
Radiotherapy - improve survival and symptoms - target : break DNA by ionization, free radical production - dividing cell : M (mitotic) and G2 phase - Photon Chemotherapy

Metastatic tumors

Epidemiology
Incidence varies among tumor type Lung 18-63% Breast 20-30% Melanoma 18-90% Ovarian 3% Prostate 1% Supratentorial : cerebral blood flow

GI/GU tumors : posterior fossa Lung : most common without primary

Pathophysiology
Hematogenous spread (tumor emboli) Gray-white junction (narrowing caliber of vessels) Enlarge in a spherical fashion, Central necrosis, edema Intratumoral hemorrhage : melanoma, choriocarcinoma, RCC, NSCLC

Management
Supportive care : corticosteroid and AEDs AAN recommends against prophylactic AEDs in brain metastasis without seizure Exception - metastatic melanoma - motor cortex - concomitant parenchymal and leptomeningeal brain metastasis

Management
Radiotherapy Surgery : single brain metastasis + absent or controlled systemic cancer Chemotherapy

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