Tumor Pada Anak
Transcript of Tumor Pada Anak
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TUMOR PADAANAK
Dr. H. Adam Suyadi, SpB, MMFakultas Kedokteran
Universitas Islam Indonesia
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Pasien anak diklasifikasikan berdasar umur
sebagai berikut:
Neonate or newborn : birth to 1 month of age; however,
a premature infant may remain in this category until
about 3 months of age
Infant : up to 1 year of age
Toddler : 1 to 3 years of age
Preschooler : 3 to 6 years of age
School-aged child : 6 to 11 years of age
Teenager ( adolescent ) : 11 to 18 years of age, many
hospi-tals consider 16 years as the upper age limit.
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CHILDHOOD CANCER
THE MOST COMMON CHILDHOOD CANCER :Leukemia, brain tumor, Hodgkins and non Hodgkins
lymphoma, and solid tumors.
SOLID TUMOR OF CHILDHOOD :
Neuroblastoma, nephroblastoma (Wilms` tumor),rhabdomyosarcoma, hepatoblastoma andhepatocellular carcinoma.
TERATOMA
HAMARTOMA
CHORISTOMA
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MEDICAL PROBLEM ?
SURGICAL PROBLEM ?
ELECTIVE ?
EMERGENCY ?
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Penyebab Distensi Abdomen
Massa abdomen :
Remembered by using the letter “F” six times :
Fetus
Flatus
Faeces
Fat
Fluid ( free and encysted),
Fibroids and other solid tumor
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Large solid tumors such as : fibroids,enlarged liver/spleen, polycystic kidneys, retroperitoneal sarcomas
Abdominal mass : Intra or Retroperitoneal
Ax: Normal passage of stool, Px: Ballotement, shiftingdullness, fluid thrill and fluctuates
X ray`s : BNO & IVP, Colon inloop, USG,Scintigrafi -MRI: colon ventromedial sites
Tumor marker : VMA, HVA – Neuroblastoma, AFP,β- HCG, LDH-1 – Germ cell tumor
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Biopsies and Surgical Procedures
Biopsy Techniques : Solid-tumor biopsies dividedinto excisional biopsies and incisional biopasiesincluded Fine Needle Biopsies (FNB)
Lymph Node Biopsies and Disections : Sentinel
Node Scintigraphy detection in Inguinal andRetroperitoneal Lymph Nodes
Primary Excisions or Exterpation
Debulking of Pediatric Solid Tumors
Secondary Surgery Residual tumor at thePrimary Site, and Second-Look Procedures hasremained controversial
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FIGURE TUMOR 1
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FIGURE TUMOR 2
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FIGURE TUMOR 3
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Figure Tumor 4
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Sejarah
Tumor Wilms:
- deskripsi awal: Max Wilms
- tumor embrional primitif ginjal (nefroblastoma)
- menejemen terapi terus berkembang-angka ketahanan hidup optimal
-multimodal terapi: operasi, kemoterapi
(preoperatif dan postoperatif), dan radiasi
-penelitian clinical trial:NWTSG (USA) dan SIOP (Eropa)
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Insidensi
- 6-10 % dari semua tumor ganas anak
- 90-95 % tumor ginjal anak
- USA : 800 kasus/tahun
NWTSG: 8 : 1000.000 penduduk
- Umur rata-rata : 2-4 tahun
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Gambaran tumor
- makros: besar, bulat, warna
putih kelabu, konsistensi lunak-
keras, fokus perdarahan ,nekrosis dan kadang kalsifikasi
- mikros (PA):
1. sel blastema tak berdiferensiasi
2. epitel berdeferensiasi: tubulus
dan glumerolus primitif
3. jaringan ikat (stroma)
kadang-kadang: otot lurik, otot polos,
lemak dll
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WILM’S Tumor
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Wilms`tumor
( Nephroblastoma )
Tumor ginjal padat yang sering dijumpai pada anak dibawah 10 tahun. 10% keganasan pada anak
10% merupakan lesi bilateral
Ditemukan sama banyak pada kedua jenis kelamin
1% ditemukan familial
Diturunkan secara dominan autosomal Incidence : 6% all of cases ( Bilateral tumor 4-6% )
The first described by Max Wilms in 1897
Relation with other congenital diseases : aniridia,hemihypertrophy,cryptorhysmus, hipospadia, pseudohermaphroditisme, disgenesis gonad, neurofibroma tosis, Beckwith-
Wiedeman Syndrome, Denis-Drash Syndrome, Perlman`s Syndrome,Klippel-Trenaunay Syndrome
DNA markers : 11p13, 11p15
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Wilms`Tumor
Clinical presentation
Diagnosis umumnya pada anak umur 1-4 tahun.Tersering pada umur 3 tahun.
Smooth, round, non-tender/ asymptomatic
abdominal mass, clearly limited seldom pass tomidline
Hematuria (20%), hypertension (20%), anorexia,
fever, weight loss (10% ), polycythemia
Subcapsulated hemorrhage
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Wilms` tumor
Examinations
BNO : calcification
Thorax x ray`s: pulmonary metastasis ( 85%)
IVP : space occupying lesion intra renal and contra
lateral functional renal USG : solid or cystic, identifying intravascular extensi-on into the renal vein (thrombus) and inferior vena cava
CT : pulmonary metastasis, enlarge tumor limited
other viscus, displaced the collecting system medially Echocardiography : atrial tumor extension
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Wilms`tumor
Pathology
The most important predictors of survival
Favorable histology (95%) respond quite well
to chemotherapy, classified elements: blastema, epithelia,
mixed, cystic, and glomelural form Unfavorable histology remaining 10% classified
as anaplastic tumors, sarcomatous, clear cell,
rhabdoid renal tumors, are treated unfavorable
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Tingkat keganasan
1. Favorable Histologis (FH)
-blastema, epitel dan stroma
-90 % kasus
-ketahanan hidup 2 tahun : 90 %
2. Unfavorable Histologis (UH)
-anaplasia (lokal/difus)
-10 % kasus
-ketahanan hidup 2 tahun : 10 %
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Wilms
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Wilms`tumor
Staging and survival
The National Wilms`Tumor Study-5 staging
system
Stage % Survival %
I 23 I 95II 23 II 90
III 23 III 80
IVV
105
IVV
60-
Treatment : Nephrectomy, Chemoterapy,rare radiation
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Tingkat penyebaran (stadium)
NWTS V (klinikopatologis=ditentukan saat operasi &
dikonfirmasi PA):
I: terbatas pada ginjal, kapsul utuh, dapat dieksisi seluruhnya,invasi tumor pada hilus (-), metastase (-)
II: meluas kepermukaan ginjal, invasi tumor pada hilus (+/-),masih dapat dieksisi seluruhnya
III:menyebar pada abdomen, infiltrasi ke organ abdomen atauorgan vital (+), limfonodi hilus dan paraorta (+), tidak dapat
dieksisi seluruhnyaIV:metastase hematogen atau limfogen keluar rongga abdomen
V: bilateral
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Pemeriksaan penunjang
- Laboratorium: darah rutin, fungsi ginjal, hati, analisa urin
- Radiologi: rontgen thoraks, IVP,USG, CT scan, MRI
CT scan sensitifitas >
spesifisitas <
menilai asal, batas-batas
dan penyebaran tumor
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Penatalaksanaan
- Standar: multi modal terapi: operasi, kemoterapi, dan
radiasi
- Operasi (standar NWTSG):
tujuan: a. diagnosis (surgical staging) ; lap. eksplorasi
b. terapi ; lap. eksplorasi + radikal nefrektomi
metode: a. operable/resectable; lap. eksp. + radikalnefrektomi
b. inoperable/unresectable; lap. eksp + biopsi
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Kemoterapi
- Tujuan: memaksimalkan membunuh tumor (tumor Wilms; kemosensitif)
- Metode:a. preoperatif (neoadjuvan)
-ukuran tumor mengecil; reseksi mudah,
resiko metastase <, resiko bedah lain <
-stadium III-IV (unresectable)
-sebelum reseksi; kontroversi ?
NWTSG: lap. ekspl. biopsi
SIOP: tanpa lap. ekspl. biopsi
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- Metode: b. postoperatif (adjuvan)
-mencegah mikrometastase bermanifest
-stadium I-V
-setelah lokal terapi (reseksi/nefrektomi)
-obat: sesuai staging & PA
- Radioterapi
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Willm’s Tumor
(Nephroblastoma)
Prognosis ;
- PA
- stadium
- ukuran tumor
- umur
- modalitas terapi
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Neuroblastoma Embryonal tumor of neural crest origin, any site in the
sympathetic nervous system includingthe brain
paraaortic sympathetic ganglia (24%)
neck (3%)
mediastinum (20%)
pelvis (3%)
adrenal medulla (50%)
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Incidence : 1:10000 life birth, more
frequently in male (2:1)
More than 25% of cases are diagnosed in the
first year of life, 50% of cases by age 2 years,
and 90% by 8 years of age
Unique characteristic spontaneous regression
N bl t
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Neuroblastoma
Clinical presentation
Abdominal firm mass and irreguler, cross midline Clinical manifestation dependent on the primary tumor,
encroachment on surrounding structures, and elaboration of
the metabolites from tumor
May present with a neck mass, Horner`s syndrome, leg paindue to metastases, respiratory distress, paraplegia, abdominal
distension due abdominal mass (60%)
Hypertension (1/3 cases), malaise, weight loss, fever,
anorexia, intractable watery diarhea and hypokalemia-dehydration due vasoactive intestinal peptide (VIP)
N bl t
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Neuroblastoma
Pathology and evaluation
Ninety- five percent of the tumor secrete catecholamine meta-
bolites homovanilic acid (HVA),and vanillylmandelic acid
(VMA) in the urine
Shimada classification system for favorable and unfavorable
histology: MKI = mitotic-karyorhectic
Favorable Unfavorable
Stroma-rich Well-differentiated
Intermixed
Nodular
Stroma- poor
Age < 18 months MKI < 200/ 5000 MKI < 100/ 50000
Age 18-60 months MKI > 100/5000
Differentiated
MKI < 100/ 50000
Undifferentiated
Age > 5 years None All
N bl t
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Neuroblastoma
Staging and Treatment
Evans Staging System
Stage Description
I Tumor confined to organ of origin
II Tumor extends beyond organ of origin but does not cross
the midline, unilateral lymph nodes may be involved
III Tumor extends beyond midline bilateral lymph nodes
maybe involved
IV Distant metastases (skeletal, other organs, soft tissues,
distant lylmph nodes)
V Would be stage I or II, remote disease confined to
liver, subcutaneous, bone marrow without evidence
bone cortex involment
Treatment : Operation and chemotherapy
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Neuroblastoma
• Tumor ganas anak
• Berasal dari sel ganglion muda susunan saraf
simpatik
• Mulai dari leher sd panggul
• Retroperitoneal perut atas
•
40% dari anak ginjal• < 2 th (50%), jarang >5 th
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• Gejala dini jarang muncul besar dan
metastasis saat diagnosis
•
Metastasis : subkutis, kel limfe regional, hati danotak
DD : Tumor Wilms
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Penanganan
• Eksisi
• Metastasis Kemoterapi
• Tumor besar: kombinasi radioterapi dan kemoterapiprabedah
Prognosis :
• Bergantung usia dan metastasis
• < 2 th + metast regional, ketahanan hidup 2 th :100%
• Regresi spontan (jarang) khas
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Adrenalectomy
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Neuroblastoma metastatik
• Pada bayi dan anak dari medula anak ginjal
tulang vertebra, tengkorak dan tulang
panjang
• Vinyl Mandelic Acid (VMA) / katekolamin
khas pada urin
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Terapi
• Kemoterapi
• Radiasi lokal
• analgesic
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Rhabdomyosarcoma
•Tipe juvenil pada anak-anak
• Predileksi : seluruh tubuh, kepala urogenital
• Tempat tumbuh : jaringan otot
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Teratoma and Germ cell tumor
Incidence : to be 1 in 20.000 to 40000 livebirths
Originate early in embryonic cell division
Tumours consisting of multiple tissues type all three
embryonic cell layers i.e. ectoderm, mesoderm,endoderm Anatomical variaty locations of teratomas but most arise in
a para axial or midline position
The most commonly diagnosed tumour in the neonate is the
sacrococcygeal teratoma
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TERATOMA
• Teratoma Sacrocoxygeal Teratoma intraabdominal
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• Teratoma merupakan kasus yang jarang.
• Insidensi 1 : 40.000 kelahiran hidupdengan teratoma sakrokoksigeus sbg
bentuk tersering ( 45-65%).
• Deteksi prenatal dgn USG,polihidroamnion, plasentomegali, dan
umur kehamilan 30 minggu prognosis
buruk.
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• Diagnosis : Pemeriksaan fisik, Foto toraks,
Foto polos Abdomen, USG, CT Scan, MRI.
• Bila tumor terletak retrorektal dpt terjadi
konstipasi.
• Eksisi komplit termasuk koksigeus penting
untuk mencegah kekambuhan.
• Angka kekambuhan 37 %.
• Bila hasil PA jinak tidak ada pengobatan
lanjut.
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• Teratoma sakrokoksigeal : tumor tersering
pada neonatus.
• Berasal dari 3 lapisan germinal embrional,
yaitu, ektoderm, mesoderm, endoderm.
• 80 % diderita oleh bayi perempuan
dengan perbandingan laki-laki dan
perempuan 4:1
• Dikelompokkan menjadi 2 tipe: Jinak atau
Matur dan ganas atau imatur.
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• Teratoma matur merupakan bentuk yang
terbanyak : 68 % pada neonatus.
• Bentuk matur biasanya kistik sedangkan
imatur biasanya padat.
•
Tumor biasanya berasal dari sakrum bagianposterior, bila pertumbuhan ke posterior
akan membentuk massa eksternal.
• Bila meluas ke anterior akan menekan
rektum mengakibatkan konstipasi.
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• Klasifikasi menurut The American
Academy of Pediatrics Surgical Section :
1. Tipe I : Sebagian besar tumor letak
eksternal dgn mini komponen presakral.
2. Tipe II: Tumor letak eksternal tetapi dgn
perluasan dominan ke pelvis
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3. Tipe III : Sedikit manifestasi eksternal,
dominan intraabdi\ominal
4. Tipe IV : Tanpa manifestasi
eksternal.
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• Diagnosis : Pemeriksaan fisik, Foto tulang
belakang, Toraks, Abdomen.
• USG, CT Scan, MRI merupakan pemeriksaan
penunjang yang penting untuk menilai asal
tumor dan perluasannya.
• Angiografi : Menilai arteri utama yang
memvaskularisasi tumor.
• Myelografi : digunakan untuk menilai
perluasan ke kanalis spinalis.
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Terapi Bedah :
•Tipe I dan II menggunakan insisi Chevronatau sagital.
•Tipe III dan IV : membutuhkan insisi
transversal abdominal bawah sebagai insisitambahan.
•Eksisi komplit tumor, ligasi a. sakralis media
dan eksisi koksigeus
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• Bila hasil PA jinak tidak ada pengobatan
lanjut,
• Bila ganas : kemoterapi cisplatin,
bleomicin, dan vinblastin.
h
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V. KASUS : Bayi perempuan 2 hari.
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Gambaran CT Scan
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• Hasil Operasi.
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•
Teratoma sakrokoksigeal biasanya tampaksebagai massa pada bidang mediana yangtimbul dari daerah sakrokoksigeal.
• Obstruksi usus (ileus) bisa terjadi bilaperluasan ke anterior dan menekanrektum.
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Leukaemia
• Leukaemia occurs when the bone marrowproduces abnormal numbers of immature whiteblood cells called blast cells. As so many
abnormal cells are being produced, the marrowcannot make enough normal white blood cellsto fight infection, red blood cells to carryoxygen and platelets that help in clotting. As aresult, the symptoms of leukaemia include
recurrent fevers, loss of energy and appetite,pallor and easy bruising.
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Brain Tumours
• The symptoms will vary depending on thelocation of the tumour and the age of the child.In a young baby, the head may rapidly increase
in size. Older children may have headaches,vomiting and drowsiness due to increasedpressure on the brain by the growing tumour.The child may develop weakness, unsteadinesswhen walking, clumsiness, double vision and
squinting if certain parts of the brain areaffected by the tumour.
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Lymphoma
•
These are tumours that start in the lymph
glands. The symptoms include swellings
in the neck, armpit, groin, chest andabdomen, which are where lymph glands
are located. There may also be recurrent
fevers, pallor and loss of weight andappetite.
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• Retinoblastoma
This is a cancer of theeye and usually occurs
in very young children
under 2 years of age.
They may present
with squinting or a
white mass seen
through the lens of
the eye.
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Surgery
•
Surgery is required for most solid
tumours. However, if the initial position
or size of the tumour makes theoperation a high-risk procedure,
chemotherapy or radiotherapy may be
given first to reduce the size of thetumour.
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Radiation Therapy
• Radiation destroys cancer cells by injuringtheir ability to divide. Special equipment
directs rays to the tumour site for a fewminutes at a time. This is done 5 times aweek for 2 to 6 weeks depending on thetype of tumour. Side effects include skin
irritation and pigmentation, which areusually temporary.
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Chemotherapy
• This involves the use of drugs that interfere with celldivision and stop the growth of tumour cells. Theycirculate throughout the body and can kill cancer cellsfar away from the original tumour site.
•
This is the mainstay of leukaemia therapy. Somechemotherapy drugs are given by injection while otherscan be taken orally.
• Side effects include hair loss, nausea and vomiting, lossof appetite, mouth ulcers and increased risk of
infection. However, steps can be taken to prevent orreduce them.
Bone Marrow Transplantation
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Bone Marrow Transplantation
• This is used mainly for high-risk leukaemia orrelapsed leukaemia, but can be used to treatother types of cancer as well.
• High doses of chemotherapy with or without
radiotherapy are given to kill cancer cells.However, the body’s normal blood stem cellsare also destroyed by the intensive treatment.Healthy blood stem cells from the child’s ownbone marrow or from a donor are then
transplanted into the body to replace thedestroyed normal cells.
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TERIMAKASIH