Man Bahadur Paudyal
Introduction IClassification:
A. Based on origin of tumor1. Primary 2. Secondary (Metastatic) B. Based on nature of tumor1. Benign: well-encapsulated, slow-growing non-infiltrative, low tendency to invade 2. Malignant: non-encapsulated, rapid-growing highly infiltrative.
Introduction IIOccurrence
CNS Tumor: 10% of all tumors Brain (80%) Spinal Cord (20%)
Ultimate outcome: increased ICP Mass effect tumor perifocal edema intratumoral hge Direct compressioninfiltration CSF pathway obstructionFocal effects CN deficits
Clinical Manifestation
Depends on the location of the tumor.With supratentorial tumors:
1. Seizure2. Mental changes (memory loss, behavior changes)3. Focal deficits(visual field deficit, paralysis)
With infratentorial tumors:1. CN palsies2. Cerebellar signs (ataxia, dysmetria, tremor, nystagmus, diadodiskinesia)3. Obstructive hydrocephalus
Clinical Syndromes Frontal lobe syndrome
1) Mental/ personality changes2) Incontinence3) Speech disorders4) Paralysis
Temporal lobe syndrome1) Aphasia2) Psychomotor seizures3) Visual field changes (hemianopia)
Parietal lobe syndrome I
I. With dominant hemisphere1) Gerstman’s syndrome
a) agraphia without alexia (can read but not write)
b) left to right confusionc) digital agnosia ( can not name
fingers)d) acalculia (can not do simple
calculation)2) Language disorder3) Tactile agnosia4) Ideomotor apraxia
Parietal Syndromes II
II. With non-dominant hemisphere1) Topographic memory loss2) Anosognosia3) Dressing apraxia
III. With either1) Focal seizures2) Agnosia3) Sensory changes4) Dyslexia
General Clinical Manifestation1. Severe progressive headache, worst in morning
2. Vomiting, projectile, without prenauseate phase3. Seizures (generalized/focal)4. Altered sensorium5. Changes in mentation6. Abnormal sensations7. Increased ICP8. Papilledema9. Central herniation with brainstem dysfunction10. Compression of brain parenchyma/blood vessels/CSF
path(1) Ischemia (2) Infarction (3) Hydrocephalus(4) Edema (5) Hemorrhage
11. Suture separation in children < 5 yr12. Bony erosion
Classification of CNS TumorIn adults, the majority are
supratentorial and of metastatic type; whereas in children, infratentorial tumors are more common.
Common Pediatric Intracranial Tumors
I. MedulloblastomaII. EpendymomaIII. Cerebral AstrocytomaIV. Cerebellar AstrocytomaV. Brainstem Glioma (BSG)VI. Craniopharyngioma (CP)
MedulloblastomaMost common malignant pediatric brain tumor (15 ~ 20%)Arises from cerebellar vermis & apex of IV ventricle roofPredispose to early obstruction of CSF pathwaysCSF seedlings
CT-scan: sold midline enhancing lesion
MRI:Banana signHighly radiosensitive/ moderately chemosensitive
TreatmentSurgery Suboccipital craniectomy+Excision ShuntVentriculostomy Definitive surgeryPostoperative radiation/ chemotherapy
PrognosisPoor prognosis Younger age < 4 yrDisseminationUnable to perform total resectionHistological differentiationWithout treatment, survival 1 ~ 2 yrsWith treatment, 5 & 10 yr survival 56% & 43%
EpendymomaUsually benign but may be inoperable due to
locationIntracranial location is usually infratentorial and in
spine, especially in filum teminalis (myxopapillary)
May seed along CSF pathways/ occur along neuraxis
CT/MRI: commonly calcified, inhomogeneous on T1WI and exophytic component is high signal in T2WI
TreatmentSurgery & postoperative radiation
PrognosisWorse due to propensity to invade obex5 yr survival 41%
Cerebral AstrocytomaTypes:
Low gr ( I + II ): less malignant better prognosisHigh gr ( III+IV ): highly malignant poor outcome
Usually involve:Frontal Basal ganglia ThalamusMidline structures
Butterfly glioma: arising from corpus callosum with bifrontal lobe involvement
Arises from neuro-ectodermal tissueTreatment: Surgery/Biopsy
Postoperative radio/chemotherapyPrognosis: Average survival < 1 yr in high grade
IV. Cerebellar astrocytoma Usually benign & cystic Usually present during 2nd decade Usually involves pons, midbrain & medulla Does not seed along CSF pathway
Pilocytic typeoccur in younger age & have better prognosisRadiographically, cystic with mural nodulePathologically, compact/loose astrocytes with Rosenthal fibres
Treatment: Surgery + Postoperative radiotherapy
Prognosis: Long-term survival possible with combined
V. Brainstem Glioma (BSG)
Slow-growing & highly malignant with poor prognosis
Usually presents with multiple CN palsies & long tract signs
Treatment: Surgery + postoperative radiotherapy
Prognosis:With RXT, survival 4 yr Without RXT, only 1 yr
Craniopharyngioma (CP) Benign suprasellar lesion Arises from rakhte’s remnants May be prechiasmatic/retrochiasmatic Causes pituitary dysfunction, visual field deficits CT/MRI: almost all have solid & cystic
componentsSpoke & Wheel appearance
Treatment: Surgery: Subtemporal/subfrontal/transsphenoidal Postoperative XRT
Prognosis: Favorable if totally resected
Common intracranial tumors in adults I. GliomaII. MeningiomaIII. Vestibular Schwanoma (Acoustic
neuroma)IV. Pituitary AdenomaV. Miscellaneous tumors
I. Glioma
All tumors that arise from neuroglial cells Consists of (1) Astrocytoma (2)
Oligodendroglioma
Oligodendroglioma (Oligo)1. Slow-growing & often calcified2. Frequently presents with seizures3. Occurs in cerebral hemispheres4. Fried egg appearance in LP microscopic view
Meningioma
Usually benign, slow-growing, frequently calcified & extra-axial
Commonly seen in middle-aged womenArising from arachnoid layerUsually located supratentorial along falx,
convexity/sphenoidTendency to compress than infiltrate brain
parenchymaClassic pathological findings is psammomna
bodiesUsually cured if completely removed which is
usually impossibleMay be highly vascular (angioblastic)
CT-scan: Homogeneously enhancing lesion with broad base attachment along durausually with little/ no perifocal edema
MRI: Dural tailTreatment: Surgery & Postoperative XRTPrognosis:
Favorable with total resection, maybe recurrentIn totally resected case, recurrent rate 15%With partial resection upto 85% after 5 yr
Pituitary AdenomaArises from anterior part of pituitary (Adenopypophysis)Type: Microadenoma < 1 cm Macroadenoma
Functional Non-functionalCauses
Compression of optic chiasma bitemporal hemianopiaCompression of cavernous sinus CN palsy(III, IV VI, V1,2)Endocrinologic disturbancesACTH Cushing’s diseasePRL Amenorrhea-galactorrhea syndromeGH Gigantism (Children) Acromegaly (adult)
Apoplexy: Abrupt onset of neurologic deterioration due to expanding mass as result of hemorrhageCauses visual deterioration, ophthalmoplegia, reduced MS & pituitary hypofunction
Acoustic SchwanomaBenign, usually unilateral or maybe part of MEN (bilateral)= NF2
Arises from vestibular branch of 8th CNCauses compression than infiltrationClassic signs: insidious/progressive
Early: Decreased hearing High-pitch tinnitusDizziness DysequilibriumLate:Hemifacial numbness (CN V palsy)Facial asymmetry, lidlag ( CN VII palsy)
Pathology: benign with Antoni A & BCT-scan: erosion/enlargement of IAC (bone
window)Ice cream cone
MRI: Round/oval enhancing lesion centered on IAC
Treatment: Surgery Retrosigmoid, Translabyrinthine, Subtemporal
Miscellaneous Tumors IColloid cyst
Classically occur in III ventricle blocking Foramen of MonroCauses obstructive hydrocephalusSlow-growing, benign lesionTreatment: Surgery( Trancallous/Transcortical Vs Endoscopic)VP Shunt
HemangioblastomaMost common primary intra-axial tumor in adult posterior fossaMay be part of von Hippel Lindau diseaseMay be associated with erythrocytosis
Cerebral LymphomaCT/MRI: homogeneously enhancing lesion in central gray matterFluffy cotton ball appearanceMay present with multiple CN palsiesExtremely responsive to steroids ( ghost tumors)Diagnosis highly likely if uveitis is present
Miscellaneous Tumors IIChordoma
Benign, highly recurrent, slow-growing, locally aggressiveGenerally radio/chemoresistentArises from remnants of primitive notochordCranially, found in sphenooccipital region (clivus) & in sacrococcygeal region in spine
Chordosarcoma: Arises from paramedial region
Cerebral MetastasisMost common brain tumor seen clinicallySources: Adults: Lung, breast, kidney(, GI, melanoma, thyroidChildren: Neuroblastoma, rhabdomyosarcoma, Wilm’s High grade glioma, medullo, ependymoma, pineal tumorLocation: parenchyma/leptomeninges
80% in cerebral hemispheres, mostly parietal lobe
Pseudotumor Cerebri (Benign/Idiopathic Intracranial Hypertension)Increased ICP papilledema without intracranial
mass, hydrocephalus or normal CT/MRI
Usually self-limited, easily recurring, chronicPreventable cause of blindness from optic atrophy
TreatmentFluid/salt restrictionDiamox 250 mg PO q8Lasix up to 160 mg/dSteroid Dexamethasone 4 mg PO q6
Prednisolone 40 ~ 60 mg PO qd
Surgery Serial LP, LP shuntOptic N sheath decompression
Treatment in General
I. Surgery if accessibleII. Radiation if
radiosensitiveIII. Chemotherapy if chemosensitive
Prognosis depends on type & location
Introduction
15% of primary CNS tumors Cranial:Spinal astrocytoma = 10:1 Cranial: Spinal ependymoma = 3 ~20:1
Most primary spinal tumors are benign
Compression symptoms
Types of Spinal Tumors
Extradural: (55%) Intradural Extramedullary: (40%)
Meningioma & neurofibroma Intramedullary: (5%)
Extradural Spinal Tumors
Metastatic Lymphoma, lung, breast, prostate
Primary Chordoma, neurofibroma,
osteoblastoma, hemangioma Meningioma
Intradural Extramedullary Spinal Tumor Meningioma neurofibroma
Intramedullary Spinal Tumor Astrocytoma 30% Ependymoma 30% Other 30%
Glioblastoma, dermoid, epidermoid, lipoma
Clinical Presentation
Pain Weakness Paresthesia Sphincter disturbance Other
Deformity: scoliosis/ torticollis SAH Mass
Diagnosis
Plain X-ray LP Myelogram CT MRI Angiography
Differential Diagnosis
Vascular malformation Demyelinating diseases ( MS) Transverse myelitis Paraneoplastic myelopathy