Tumor Pada Anak

7/28/2019 Tumor Pada Anak

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TUMOR PADAANAK

Dr. H. Adam Suyadi, SpB, MMFakultas 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 andhepatocellular 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, 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



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



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



WILM’S 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, pseudohermaphroditisme, 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 tomidline

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 95II 23 II 90

III 23 III 80

IVV

105

IVV

60-

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 atauorgan vital (+), limfonodi hilus dan paraorta (+), tidak dapat

dieksisi seluruhnyaIV: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. + radikalnefrektomi

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



Willm’s Tumor

(Nephroblastoma)

Prognosis ;

- PA

- stadium

- ukuran tumor

- umur

- modalitas terapi



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%)



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



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



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



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



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 danotak

DD : Tumor Wilms



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



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 hidupdengan 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 bagianposterior, 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 Chevronatau sagital.

•Tipe III dan IV : membutuhkan insisi

transversal abdominal bawah sebagai insisitambahan.

•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.

h



V. KASUS : Bayi perempuan 2 hari.



Gambaran CT Scan



• Hasil Operasi.



•

Teratoma sakrokoksigeal biasanya tampaksebagai massa pada bidang mediana yangtimbul dari daerah sakrokoksigeal.

• Obstruksi usus (ileus) bisa terjadi bilaperluasan ke anterior dan menekanrektum.



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.



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.



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.



• 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.



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.



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.



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



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.



TERIMAKASIH

Tumor Pada Anak

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