TUMOR PADA

ANAK

Dr. H. Adam Suyadi, SpB, MM

Fakultas Kedokteran
Universitas Islam Indonesia
 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.
 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 and
hepatocellular carcinoma.
TERATOMA
HAMARTOMA
CHORISTOMA
MEDICAL PROBLEM ?
SURGICAL PROBLEM ?
ELECTIVE ?
EMERGENCY ?
 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
 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, shifting
dullness, fluid thrill and fluctuates
X ray`s : BNO & IVP, Colon inloop, USG,Scintigrafi -
MRI: colon ventromedial sites
Tumor marker : VMA, HVA Neuroblastoma, AFP,
- HCG, LDH-1 Germ cell tumor
 Biopsies and Surgical Procedures

Biopsy Techniques : Solid-tumor biopsies divided

into excisional biopsies and incisional biopasies
included Fine Needle Biopsies (FNB)
Lymph Node Biopsies and Disections : Sentinel
Node Scintigraphy detection in Inguinal and
Retroperitoneal Lymph Nodes
Primary Excisions or Exterpation
Debulking of Pediatric Solid Tumors
Secondary Surgery Residual tumor at the
Primary Site, and Second-Look Procedures has
remained controversial
FIGURE TUMOR 1
FIGURE TUMOR 2
FIGURE TUMOR 3
Figure Tumor 4
 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)
 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
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
WILMS Tumor
 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, pseudo
hermaphroditisme, disgenesis gonad, neurofibroma tosis, Beckwith-
Wiedeman Syndrome, Denis-Drash Syndrome, Perlman`s Syndrome,
Klippel-Trenaunay Syndrome
DNA markers : 11p13, 11p15
 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 to
midline
Hematuria (20%), hypertension (20%), anorexia,
fever, weight loss (10% ), polycythemia
Subcapsulated hemorrhage
 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
 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
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 %
Wilms
 Wilms`tumor
Staging and survival
The National Wilms`Tumor Study-5 staging
system
Stage % Survival %
I 23 I 95
II 23 II 90
III 23 III 80
IV 10 IV 60
V 5 V -

Treatment : Nephrectomy, Chemoterapy,rare radiation

 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 atau
organ vital (+), limfonodi hilus dan paraorta (+), tidak dapat
dieksisi seluruhnya
IV:metastase hematogen atau limfogen keluar rongga abdomen
V: bilateral
 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
 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. + radikal
nefrektomi
b. inoperable/unresectable; lap. eksp + biopsi
 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
- Metode: b. postoperatif (adjuvan)
-mencegah mikrometastase bermanifest
-stadium I-V
-setelah lokal terapi (reseksi/nefrektomi)
-obat: sesuai staging & PA
- Radioterapi
Prognosis ;
- PA
- stadium
- ukuran tumor
- umur
- modalitas terapi

Willms Tumor
(Nephroblastoma)
 Neuroblastoma
Embryonal tumor of neural crest origin, any site in the
sympathetic nervous system including
the brain
paraaortic sympathetic ganglia (24%)
neck (3%)
mediastinum (20%)
pelvis (3%)
adrenal medulla (50%)
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
 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 pain
due 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)
 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 Nodular
Intermixed
Stroma- poor

Age < 18 months MKI < 200/ 5000 MKI < 100/ 50000

Age 18-60 months MKI > 100/5000 MKI < 100/ 50000
Differentiated Undifferentiated
Age > 5 years None All
 Neuroblastoma
Staging and Treatment
Evans Staging System
Stage Description

I Tumor confined to organ of origin

Tumor extends beyond organ of origin but does not cross
II
the midline, unilateral lymph nodes may be involved

III Tumor extends beyond midline bilateral lymph nodes

maybe involved
Distant metastases (skeletal, other organs, soft tissues,
IV
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

 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
 Gejala dini jarang muncul besar dan
metastasis saat diagnosis
Metastasis : subkutis, kel limfe regional, hati dan
otak
DD : Tumor Wilms
 Penanganan
Eksisi
Metastasis Kemoterapi
Tumor besar: kombinasi radioterapi dan kemoterapi
prabedah

Prognosis :
Bergantung usia dan metastasis
< 2 th + metast regional, ketahanan hidup 2 th :
100%
Regresi spontan (jarang) khas
Adrenalectomy
 Neuroblastoma metastatik
Pada bayi dan anak dari medula anak ginjal
tulang vertebra, tengkorak dan tulang
panjang
Vinyl Mandelic Acid (VMA) / katekolamin
khas pada urin
 Terapi
Kemoterapi
Radiasi lokal
analgesic
 Rhabdomyosarcoma
Tipe juvenil pada anak-anak
Predileksi : seluruh tubuh, kepala urogenital
Tempat tumbuh : jaringan otot
 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
 TERATOMA

Teratoma Sacrocoxygeal Teratoma intraabdominal

 Teratoma merupakan kasus yang jarang.
Insidensi 1 : 40.000 kelahiran hidup
dengan teratoma sakrokoksigeus sbg
bentuk tersering ( 45-65%).
Deteksi prenatal dgn USG,
polihidroamnion, plasentomegali, dan
umur kehamilan 30 minggu prognosis
buruk.
 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.
 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.
 Teratoma matur merupakan bentuk yang
terbanyak : 68 % pada neonatus.
Bentuk matur biasanya kistik sedangkan
imatur biasanya padat.
Tumor biasanya berasal dari sakrum bagian
posterior, bila pertumbuhan ke posterior
akan membentuk massa eksternal.
Bila meluas ke anterior akan menekan
rektum mengakibatkan konstipasi.
 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
3. Tipe III : Sedikit manifestasi eksternal,
dominan intraabdi\ominal
4. Tipe IV : Tanpa manifestasi
eksternal.
 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.
 Terapi Bedah :
Tipe I dan II menggunakan insisi Chevron
atau sagital.
Tipe III dan IV : membutuhkan insisi
transversal abdominal bawah sebagai insisi
tambahan.
Eksisi komplit tumor, ligasi a. sakralis media
dan eksisi koksigeus
 Bila hasil PA jinak tidak ada pengobatan
lanjut,
Bila ganas : kemoterapi cisplatin,
bleomicin, dan vinblastin.
V. KASUS : Bayi perempuan 2 hari.
Gambaran CT Scan
 Hasil Operasi.
 Teratoma sakrokoksigeal biasanya tampak
sebagai massa pada bidang mediana yang
timbul dari daerah sakrokoksigeal.

Obstruksi usus (ileus) bisa terjadi bila

perluasan ke anterior dan menekan
rektum.
 Leukaemia

Leukaemia occurs when the bone marrow

produces abnormal numbers of immature white
blood cells called blast cells. As so many
abnormal cells are being produced, the marrow
cannot make enough normal white blood cells
to fight infection, red blood cells to carry
oxygen and platelets that help in clotting. As a
result, the symptoms of leukaemia include
recurrent fevers, loss of energy and appetite,
pallor and easy bruising.
 Brain Tumours

The symptoms will vary depending on the

location 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 increased
pressure on the brain by the growing tumour.
The child may develop weakness, unsteadiness
when walking, clumsiness, double vision and
squinting if certain parts of the brain are
affected by the tumour.
 Lymphoma

These are tumours that start in the lymph

glands. The symptoms include swellings
in the neck, armpit, groin, chest and
abdomen, which are where lymph glands
are located. There may also be recurrent
fevers, pallor and loss of weight and
appetite.
 Retinoblastoma
This is a cancer of the
eye 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.
 Surgery

Surgery is required for most solid

tumours. However, if the initial position
or size of the tumour makes the
operation a high-risk procedure,
chemotherapy or radiotherapy may be
given first to reduce the size of the
tumour.
 Radiation Therapy

Radiation destroys cancer cells by injuring

their ability to divide. Special equipment
directs rays to the tumour site for a few
minutes at a time. This is done 5 times a
week for 2 to 6 weeks depending on the
type of tumour. Side effects include skin
irritation and pigmentation, which are
usually temporary.
 Chemotherapy
This involves the use of drugs that interfere with cell
division and stop the growth of tumour cells. They
circulate throughout the body and can kill cancer cells
far away from the original tumour site.
This is the mainstay of leukaemia therapy. Some
chemotherapy drugs are given by injection while others
can be taken orally.
Side effects include hair loss, nausea and vomiting, loss
of appetite, mouth ulcers and increased risk of
infection. However, steps can be taken to prevent or
reduce them.
 Bone Marrow Transplantation

This is used mainly for high-risk leukaemia or

relapsed leukaemia, but can be used to treat
other types of cancer as well.
High doses of chemotherapy with or without
radiotherapy are given to kill cancer cells.
However, the bodys normal blood stem cells
are also destroyed by the intensive treatment.
Healthy blood stem cells from the childs own
bone marrow or from a donor are then
transplanted into the body to replace the
destroyed normal cells.
TERIMAKASIH

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