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TUMOURS OF THE CENTRAL

NERVOUS SYSTEM

Dr.Apriyanto SpBS.Mkes
BAB I
PENDAHULUAN
Tumor otak atau tumor intrakranial neoplasma
atau proses desak ruang (space occupying lession
atau space taking lession) yang timbul di dalam
rongga tengkorak.
Data statistik insidens tumor otak di Amerika :
16,5 per 100.000 populasi per tahun, dimana
17.030 kasus tumor primer baru & separuh
sisanya 17.380 lesi metastasis
EPIDEMIOLOGY
InUS,atleast29.000newcasesofprimary
malignantandbenigntumorsarediagnosedper
years.
Basedonresearch,Centralbraintumorregistry
ofUS20052006insidenceofbraintumorinUS
around14,8/100.000ofallpeopleperyear.
Theoverall,insidenceofbraintumorsare
increasinginelderly
male<female

InIndonesia,thereisnoresearch
Anatomi Otak
2.2 DEFINISI TUMOR OTAK
Tumor otak adalah suatu lesi ekspansif yang
bersifat jinak (benigna) ataupun ganas
(maligna) membentuk massa dalam ruang
tengkorak kepala (intra cranial) atau di
sumsum tulang belakang (medulla spinalis).
2.2 Etiologi Tumor Otak
1. Herediter
2. Sisa-sisa Sel Embrional (Embryonic Cell
Rest)
3. Radiasi
4. Virus
5. Substansi-substansi Karsinogenik
2.2 Klasifikasi Tumor Otak
KlasifikasitumorOtakberdasarkanWorldHealth
Organization(WHO):
General
o Increased intracranial pressure
o Seizure
o Intracranial bleeding
o Focal neurologic deficits
o Obstruction of flow of CSF
o Pituitary dysfunction
Cerebral Tumors
o Headache
o Vomiting unrelated to food intake
o Changes in visual fields and acuity
o Hemiparesis or hemiplegia
o Hypokinesia
o Decreased tactile discrimination
o Seizures
o Changes in personality or behavior
Brainstem tumors
o Hearing loss (acoustic neuroma)
o Facial pain and weakness
o Dysphagia, decreased gag reflex
o Nystagmus
o Hoarseness
o Ataxia (loss of muscle coordination) and dysarthria (speech
muscle disorder) (cerebellar tumors)
Cerebellar tumors
o Disturbances in coordination and equilibrium

Pituitary tumors
o Endocrine
dysfunction
o Visual deficits
o Headache
Frontal Lobe
o Inappropriate behavior
o Personality changes
o Inability to concentrate
o Impaired judgment
o Memory loss
o Headache
o Expressive aphasia
o Motor dysfunctions
Parietal lobe
o Sensory deficits
Paresthesia
Loss of 2 pt discrimination
Visual field deficits
Temporal lobe
o Psychomotor seizures temporal lobe-judgment,
behavior, hallucinations, visceral symptoms, no
convulsions, but loss of consciousness
Occipital lobe
o Visual disturbances
1. TUMOR EPITHELIAL
1. Tumor Glial
a. Astrositoma
ThesecondcommontumourinCNSafter
glioblastoma.
Approximately1113%fromallofCNStumours

Averageagesfrom35to45year

Male>female

a. Grade Astrositoma :
Grade I (Astrositoma Pilositik)

Grade II (Astrositoma Difus)

Grade III (Astrositoma Anaplastik) dan

Grade IV (Glioblastoma Multiforme)


CLINICALSIGN
Seizuregeneralorfokal
Headache

Vomitus

Paresisfacialorextremities

Visualdefisit

Disfasia,diplopia,vertigo,hemianopsia
homonimus
unconciousness
Gambar 2.3 Gambaran Gambar 2.4 Gambaran CT-
Histopatologi Low Grade Scan Low Grade
Astrocytoma 11 Astrocytoma 11
*CONTOHKASUS

Gambar 2.5 Gambaran MRI T1 Axial. Preoperatif


dan postoperatif pasien Laki-laki, 43 tahun. 11
TREATMENT
Surgery
Radiation

Kemoterapy
b. Oligodendroglioma tumour
Peak insidence are the fourth and the sixth
decades
Slowly growing, seizure occur approximately
32% pf the patient
Male>female
*CONTOHKASUS

Gambar 2.6 Gambaran MRI T1 Axial.


Preoperatif dan postoperatif pasien Perempuan,
28 tahun. 11
c. Ependimoma tumour
2-8% of all all CNS neoplasms and in
childhood, are the third most common CNS
tumours
Site tumour : childhood Infratentorial

Adult Supratentorial
.
CLINICALSIGNANDIMAGING
<1yearsold hidrosepalus
Nuchal rigidity, lethargy, neck pain, torticalis and head
tilt
> 2 years old increasing ICP
Gambar 2.7 Gambaran Penumpukan zat Kontras
pada Tumor di Ventrikel Lateral Ependimoma12
TREATMENT
Surgery
Adjunctivetherapy>especiallyinroutine
treatmentofinfratentorialependymomaof
chilhood.
2. Tumor Non-Glial
Tumor Primitive Neuroektodermal
Supratentorial (PNET)
* Maligna embrional tumor
differenciate divergen origin from
germinal matrics of the primitive
neural tube.
CHOROIDPLEXUSTUMOR
Peakinsidenceisinthefirsttwo
decades
5070%casesoccurinadults
itsfoundmostfrequentlyinthe
lateralventricles.
CHOROIDPLEXUSTUMOR
Gambaran MRI T1 Sagital. Postkontras.
Tumor ependimoma 4th ventricel.
MEDULOBLASTOMA

The Most common malignant brain tumor


of childhood and account for 25% of all
pediatric tumor
Approximately 75% cases occur in the first
15 years.
MEDULOBLASTOMASIGNSAND
SYMTOMS
Lethargy,irritabilityandlossofappetite
MostpatientpresentwithincreasedICP

Truncalataxia,limbataxiaordysmetriaand
nystagmus.
Sixthnervepalsies.
Gambar 2.9 Gambaran MRI
Meduloblastoma di Cerebellum 13
Gambar 2.10 Gambaran Histopatologik
Sel Rosette pseudorosette pada pasien
dengan Meduloblastoma13
2.MENINGEALTUMOR
1. Meningioma
Benign tumour that origin from meningen.
Sex hormones, radiation, and chromosones play a
role pathogenesis.
PATHOGENESIS
they express progesterone reseptor, that is
why female >male.
Ussually lost part of the long arm of
chromosome 22 previous radiation
Increasing age caused failre DNA repair
mechanism
Neurofibromatosis type 2 deletion of
neurofibromin of chromosome 22
CLINICALLYSIGNANDIMAGING
Seizuremorethanhalfthepatientsand10%
haventsymptom
CTorMRIfiguredisodenseorisointensewith
brainandcalcified.Withcontrastheyenhance
homogeneouslyandbrighly
Gambar 2.11 Gambaran CT-Scan venogram
potongan koronal Meningioma di Sinus
Sagitalis Superior
Gambar 2.12 Gambaran Tekhnik
pengangkatan Meningioma11
2. Hemangioperisitoma
Tumor ini termasuk golongan tumor yang
vaskuler, dengan terapi definitifnya
adalah reseksi.
Peranan angiografi & embolisasi
meningkatkan efektifitas dan keamanan
reseksi yang dilakukan
3.TUMORSELLA
1. Kraniofaringioma
Its slow growing, benign epithelial
neoplams of the sellar region
embriologically originate from
remnant s of embryonic squamous cell
rests of an incomplete involutes
hypophyseal-pharyngeal duct.
EPIDEMIOLOGY
Incidence0,5to2cases/millionspopulationper
year
Thetumourisseeninbothadultandchildren

Peakinsidenceatage5to10yearsandbetween
ages50and60years.
CLINICALPERSENTATION
Dependonalargeextentontheorigin,direction,
degreeoftumorextensionandinvolvementof
surounding.
IncreasedICP

Visualchanges

Endocrineabnormalitiessuchasinchildren
presentwithshortstatureandretardedlinear
growth.Inadolescentswithdelayingorarresting
pubertyandadultmaycomplainlosslibido.
Mentaldisturbance
Gambar 2.13 Gambaran MRI T1 Postkontras Potongan Koronal (A)
dan Sagital (B) Tumor Kistik Selar dan Supraselar Kraniofaringioma.
Pasien mengalami penurunan penglihatan 11
MANAGEMENT
Multidisiplinaryapproach
Surgicaldependonlocation

Radiation

chemotherapy
2. Adenoma Hipofisis
10% of all intracranial neoplasms
Jenis tumor ketiga terbanyak setelah glioma dan
mengioma.
Female>male
Unknown etiology
CLINICALSIGN

Decreasedinthesecrretionofhormonesfromadeno
hypophisis
DecreasedTSH>hypothyroid

ACTH>addisondisease

LHandFSH>amenorrhea

GH>lossofnormalgrowth

>prolactin

Diabetesinsipidus

CranialnervepareseIII,IV,VI>diplopia

CranialnerveV>ipsilateralfacialnumbnessetc
Gambar 2.14 Gambaran Adenoma Hipofise Pasien
Perempuan 54 tahun dengan multinodular goiter11
Gambar 2.15 Akromegali pada Seorang
Penderita Tumor Adenoma Hipofise Gambar 2.16 Gigantisme pada
Seorang Penderita Tumor Adenoma
Hipofise
2.6 Pemeriksaan Penunjang Tumor Otak
CT Scan + Ct Scan Contras

MRI + MRI Contras

Pemeriksaan terpilih untuk mendeteksi adanya


tumor tumor intrakranial

2.7 Jenis Tumor Otak pada Lokasi Spesifik Tumor


pada Sistem Ventrikel
Tumor pada Daerah Talamus
Tumor pada Khiasma/Sella Tursika
Tumor pada daerah Pineal/Epifise
Tumor Batang Otak
Tumor Daerah Serebelum
Tumor Sudut Serebelo-Pontin
Tumor Kongenital
Tumor Metastatik pada Otak
Gambar 2.17 Gambaran MRI
Postkontras Potongan aksial Pasien
dengan Tumor Metastasis dari Renal Cell
Carcinoma
2.8 Diagnosis Tumor Otak
1. Anamnesis
2. Pemeriksaan Fisik
3. Pemeriksaan Penunjang
2.9 DIAGNOSIS BANDING TUMOR OTAK
Abses Intraserebral

Gambar 2.18 Gambaran Abses


Serebri
2.10 Penanganan Tumor Otak
Modalitas penanganan terhadap tumor otak
mencakup tindakan-tindakan:
Terapi Kortikosteroid

Terapi operatif
o Tujuan: mendapatkan diagnosa pasti dan

dekompresi internal.
Terapi konservatif

Radioterapi
Kemoterapi
Immunoterapi
2.11 Prognosis Tumor Otak
Prognosis tergantung jenis tumor spesifik.

Negara maju : diagnosa dini penanganan


tepat (pembedahan + radioterapi) 5 years
survival berkisar 50-60% dan (10 years
survival) berkisar 30-40%.
BAB III
KESIMPULAN
Tumor otak termasuk penyakit yang sulit terdiagnosa
secara dini. Secara klinis sukar membedakan antara
tumor otak yang benigna atau maligna.
Curiga tumor otak bila adanya gangguan cerebral
umum yang bersifat progresif; adanya gejala tekanan
tinggi intrakranial dan adanya gejala sindrom otak
yang spesifik
Pemeriksaan radiologi; CT-Scan, diagnosa pasti
tumor otak benigna atau maligna dengan
pemeriksaan patologi anatomi.
DAFTARPUSTAKA
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editor. Edisi ke-tiga. Jakarta: Gramedia pustaka utama; 1980. hal. 207
49.
2. Mardjono, Mahar. Proses neoplasmatik di susunan saraf. Dalam:
neurologi klinis dasar. Jakarta: PT. Dian Rakyat; 2008. hal. 390 402
3. Berttolone SJ. Tumor of the central nervous system concepts in cancer
medicine. 1982:649 59
4. Hakim A.A. Tindakan Bedah pada Tumor Cerebellopontine Angle,
Majalah Kedokteran Nusantara Vol. 38 No 3, 2005.
5. Adams and Victors. Intracranial Neoplasms and Paraneoplastic Disorders
in Manual of edisi 7, McGraw Hill, New York, 2002 : 258 63
6. Tumor Otak dalam Buku Ajar Neurologi Klinis edisi I. Yogyakarta;
Gajah Mada University Press; 1999. hal: 201 7
7. Lindsay, Kenneth W. Neurology and neurosurgery illustrated. Edinburgh
2nd ed. London: Churchill Livingstone. 1991. p. 292 340
8. Bernstein,Mark.Neurooncologythe
essentials.ThiemeMedicalPublisher;2000.p.30299
9. BlackPB.Braintumor,reviewarticle.TheNEJM.
1991(324):14712
10. Snell,RichardS.Neuroanatomiklinik.Jakarta:EGC;
2007.
11. DemonteFranco,dkk.Tumorsofthebrainandspine.
Texas:TheUniversityofTexasM.DAndersonCancer
Center;2007.
12. Kaye,AndrewH.EssentialNeurosurgery.ThirdEd.
Australia:BlackwellPublishing;2005.
13. MacDonal,Tobey.PediatricMedulloblastoma(serial
online)2012March1st(diakses21Juli2012).Diunduh
dari:URL:
http://emedicine.medscape.com/article/987886overview
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-TERIMA KASIH-

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