TUMOR PADA ANAK

Moch. Junaidy Heriyanto, dr, SpB,

FINACS

Di USA
Childhood Cancers
Brain
Tumors

Other

Leukemia

Retinoblastoma
Bone tumors
Kidney tumors

Lymphoma

Soft tissue sarcomas

Neuroblastoma

Catatan: data di USA

Leukemia paling sering (keganasan
hematologi/non-solid tumor)
Brain tumors tersering kedua
Lymphomas are the tersering ketiga
Kemudian solid tumors diluar CNS

Neuroblastoma - neural crest derived

Wilms - renal tumors and syndromes
Bone tumors
Rhabdomyosarcoma - soft tissue sarcomas

Tiga terbanyak tumor pada anakanak

Wilms tumor/nefroblastoma
Neuroblastoma
Teratoma (55% berupa sakrokoksegeal
teratoma)

CNS Tumors

Presentation
23mo female with ataxia X 1month

NB NB Emesis, in am X 2weeks
Vomiting am
Seen by PCP for GERD without improvement
No Fevers
Increasing vomiting

Test

MRI

MRI

Brain Tumors of Childhood

Heterogeneous
* Cell of origin:
glial, neural, other, combination

* Location:
posterior fossa: 50%
supratentorial: 50%
* Clinical presentation:
location
age

Brain Tumors of Childhood

Infratentorial
50%
esp < 6 y/o

Supratentorial
50%
esp > 8 y/o

Nonlocalizing Signs of Brain Tumors

Increased intracranial pressure (ICP)

Obstructed CSF flow
and hydrocephalus
Child is often asymptomatic
until critical threshold
reached

Medulloblastoma
Can grow very large
before detection

Increased Intracranial Pressure

(ICP)
Headaches, progressively worsening
Vomiting (morning)
Irritability
Papilledema
rare < 2 y/o - head can expand

Double vision with 6th nerve palsy

Head tilt
Bulging fontanel (infant)

In a young child with ? brain tumor:

Measure head circumference and observe gait

Supratentorial Tumors
Signs depend on location and age
like in adults; in addition:
Younger child:
Developmental delay or loss of milestones
Older child:
Deteriorating school performance
Personality changes
Endocrinopathies:
DI, hypothyroidism, precocious puberty

Lymphomas

Childhood Lymphomas
Signs and Symptoms depend on:
Lymphoma subtype

Hodgkins Disease (HD)

Nonhodgkins Lymphoma (NHL)
* Burkitts
* Lymphoblastic
* Anaplastic Large Cell

Location

Presentation of Hodgkins
Disease
Age: adolescents >> young child
Painless lymphadenopathy
Progresses over weeks months

Location
Cervical/supraclavicular LNS
unilateral or bilateral
Mediastinum hilum
LNs below diaphragm and spleen
Liver, lung, bone marrow

95%

Presentation of Hodgkins Disease

Systemic symptoms

Fevers
Night sweats
Weight loss
Pruritus

B symptoms
25%

Superior Mediastinal Syndrome (SMS)

Orthopnea, SOB, stridor, hypoxia
Tracheal
Bronchial
Cardiac

compression

= Oncologic Emergency

NEFROBLASTOMA = Wilms
tumor

18

Epidemologi

Tumor ginjal terbanyak pada anak

Usia 6 bulan 10 tahun
Terbanyak usia 3,5 tahun
< 10% bilateral
Nama lain Wilms tumor/karsinoma sel
embrional

19

Patologi
Berasal dari blastema, stroma dan epitel
Histopatologi dibedakan Favorable dan
Unfavorable

20

Penyebaran
Invasi langsung ke organ sekitar
Limfogen melalui kel para aorta
Hematogen melalui vena renalis ke vena
kava
85% ke paru 10% ke liver

21

Wilms tumor: Signs and

Symptoms
Abdominal mass
Often asymptomatic

Healthy appearing

2 days
before
dx

Encapsulated
mass

Wilms tumor: Signs and Symptoms

BP 25%
Mass enlarges toward pelvis

Signs and Symptoms of

Wilms tumor
Associated anomalies, syndromes 15%
Hemihypertrophy
Aniridia

WAGR syndrome
Wilms, aniridia,
ambiguous genitalia, retardation

Signs and Symptoms of

Wilms tumor
More anomalies, syndromes
GU anomalies
Denys-Drash syndrome

GU anomalies and renal failure

Beckwith-Wiedemann syndrome
Hypoglycemia
Macroglossia

Umbilical hernia
Hemihypertrophy

Gambaran Klinik

Perut membuncit
Benjolan di perut atas
Kencing berdarah
Kadang hipertensi

26

Pemeriksaan Penunjang
USG : masa padat diperut atas yang
retroperitoneal
IVP : distorsi sistem pelvikalises atau non
visualized

27

Stadium
Menurut National Wilms Tumor Study
(NWTS)

I : Terbatas pada ginjal, dapat dieksisi sempurna

II : Meluas keluar ginjal, dapat dieksisi sempurna
III : Ada sisa tumor di abdomen (dari sisa operasi)
IV : Metastase hematogen
V : Tumor bilateral

28

CT scan - Stage III Wilms Tumor

CT scan - Stage IV Neuroblastoma

Penatalaksanaan
Terapi utama : Pembedahan
Kemoterapi
Radioterapi

31

Surgery
The main responsibility of
the surgeon is to:
Remove the tumor
completely, without
spillage
Acurrately assess the
extent to which the
tumor has spread
Pay particular attention
to adequately
assessing the lymph
node involvement

Radical Nephrectomy

Radical Nephrectomy

Tumor spillage
associated with
recurrence

Role of Laparoscopy
Laparoscopic Unilateral Nephrectomy or
Partial Nephrectomy described for
treatment of Wilmstumor.
Case reports, particularily performed in
Europe where children get neoadjuvant
chemotherapy.
Currently not endorsed in US.

Diagnosa Banding
Neuroblastoma
Teratoma retroperitoneum
Kista ginjal

36

NEUROBLASTOMA

37

Epidemologi
Prevalensi : 1/10.000-30.000 kelahiran
hidup
65% berasal dari kelenjar adrenal
75% merupakan tumor abdomen
Wanita>pria
85% pada usia 6 tahun
7-10% bilateral
38

Patologi
Tumor yang muncul dari jaringan embrional
neural crest dari medula adrenal atau
simpatik nervus dari abdomen, toraks,
pelvis atau kepala dan leher

39

Penyebaran
60% ke tulang
Regional limfonodi 42%
Liver 15%

40

Gambaran klinis
dari kasus datang dengan benjolan pada
abdomen
Penurunan berat badan
Anorexia
Panas

42

Pemeriksaan penunjang
Peningkatan jumlah catecholamin,
vanilmandelic acid dan homovanilic acid
IVP didapatkan gambaran posisi ginjal
yang terletak lebih ke bawah
CT Scan

43

Staging
I : Tumor terbatas pada daerah asalnya,
limfonodi pada ipsilateral dan kontralateral II : Tumor melewati daerah asalnya, tidak
melewati garis tengah, limfonodi ipsilateral +,
kontralateral III : Tumor melewati garis tengah, limfonodi,
ipsilateral dan kontralateral +
IV : Metastase jauh
44

Penatalaksanaan
Terapi utama adalah pembedahan
Kemoterapi dan radioterapi

46

TERATOMA

47

Teratoma Sakrokoksigeal
Paling sering ditemukan pada neonatus
Insiden : 1/40.000 kelahiran
81% mengenai wanita
Sebagian besar tumor jinak
Insiden keganasan meningkat sesuai
meningkatnya umur
55% dari semua kasus teratoma

48

Patologi
Merupakan tumor bulat lonjong atau
berlobus yang terbungkus kapsul
Terdiri dari bagian yang solid atau kistik,
kadang campuran solid dan kistik
Kadang berisi organ tubuh yang matur
81% terdiri dari tipe I&II (ALTMAN)

49

 Berasal dari 3 germinal sel

Endoderm
Mesoderm
Ectoderm

50

Klassifikasi
Menurut ALTMAN
Tipe I : Tumor terlihat di luar, komponen
presakral minimal
Tipe II : Tumor terlihat dari luar, bagian
terbanyak dalam rongga pelvis
Tipe III : Tumor terlihat sedikit dari luar, bagian
dalam mencapai rongga abdomen
Tipe IV : Tumor tidak terlihat dari luar, tumor
hanya berada pada rongga presakral
51

Gambaran klinis
Tumor di daerah sakrum dan koksigeus,
berupa masa solid atau kistik yang terletak
pada garis tengah
1/5 dari kasus yang ada disertai kelainan
kongenital lain

52

Diagnostik penunjang
USG
CT Scan
AFP

53

Penatalaksanaan
Operasi merupakan terapi untuk teratoma
sakrokoksigeal

54

Bone tumors

Bone Tumors in Childhood

Age Adolescents > younger children
Signs and symptoms

Bone pain, palpable mass, motion

Often hx of sports injury (coincidental)

Osteogenic Sarcoma
Metaphyses of long bones:
Distal femur
Proximal tibia
Proximal humerus
Pelvis

Ewing Sarcoma
All bones:
Long: diaphyses
Flat
Pelvis
Skull
Ribs

Presentation of Bone Tumors

Classic X-ray
of Ewing:
Moth-eaten
lytic lesion

Classic X-ray of O.S.:

Sunburst pattern
Periosteal reaction
Soft tissue mass + calcium

Plain X-Rays are usually abnormal

Presentation of Bone Tumors

Further radiographic evaluation may help
with differential diagnosis of bone pain
Bone scan
MRI
Chest CT scan
Metastases 20%

Pelvic Ewings sarcoma

Soft tissue sarcomas

Presentation of Soft Tissue Sarcomas

Rhabdomyosarcoma most common
Age
Birth to > 20 y/o
70% < 10 y/o

Sites

Head and neck 40%

Genitourinary 20%
Extremities 20%
Trunk 10%
Retroperitoneal 10%

Signs and
symptoms
depend on
age and site

Rhabdomyosarcomas: Signs and Symptoms

Head and neck

Orbit
Proptosis
Periorbital swelling

Parameningeal
Cranial nerve palsies
Hearing loss
Chronic aural or
sinus drainage

Same patient:
S/P radiation
and chemo

3 months off Rx:

eye lashes regrown

Rhabdomyosarcomas: Signs and

Symptoms
Genitourinary

Bladder and prostate

Hematuria
Urinary obstruction

Paratesticular
Painless mass - testicle

Vagina and uterus

Abdominal mass
Vaginal mass
Vaginal bleeding or discharge

Botryoid:
grape-like

Rhabdomyosarcoma other sites

Can show up at any site and any age
6 week old

Newborn

Kelainan Bawaan

INTRODUCTION
Patient can be different :
Surgical Problem - Operations
Medical Problem - Conservative
and in Surgical Problem, divided :
Emergencies : Acute abdomen
Electif

Surgical Intestinal
Emergencies of Infancy

Gastroschisis
Omphalocele
Meckels diverticulum
Diaphragmatic hernia
Eventration of the diaphragm
Esophageal atresia and
trachea esophageal fistuls
Hypertrophic pyloric stenosis
Atresia duodenum
Pancreas annulare
Meconium ileus
Volvulus
Anorectal anomalies
Strangulated inguinal hernia
Intussusceptions
Meconium ileus
Hirschsprung

Clinical manifestation as
Abdominal acute

THE DIGESTIVE SYSTEM

Primitive gut (foregut, midgut,and hindgut)

origin from dorsal part yolk sac in fourth weeks

FOREGUT: PHARYNX, LOWER RESPIRATORY

TRACT, OESOPHAGUS, GASTER,
DUODENUM,PROXIMAL BILIARY TRACT,
LIVER,PANCREAS, BILIARY APPARATUS

MIDGUT: DUODENUM(DISTAL BILE

DUCT),JEJUNUM,ILEUM,COECUM,VER MIFORM
APPENDIX, ASCENDING CO- LON,
2/3PROXIMALTRANVERSECOLON

HINDGUT:1/3 DISTAL TRANSVERSE

COLON,DESCENT COLON, SIGMOID,
REKTUM,UPPER PART ANUS,

ANATOMI GIT

Gastric outlet
Ampulla of Vater

Peyery patchs of lymphoid

Allogaritme
Management Patient
Anamnesis: Chief complain - symptoms
History
Physical Examinations:
Physical signs
Laboratory: blood, urine etc.
X rays plain USG (non invasive)
- contras CTscan MRI
Diagnosis
Treatment
Prognosis

SYMPTOMS AND SIGNS

GASTRO-INTESTINAL TRACT

OBSTIPATIONS
DISTENSIONS
VOMITING
ABDOMINAL PAIN
ABDOMINAL SIGN

Symptoms and Signs

of Surgical Disease

Obstipation : Intractable
constipations

Constipation : Deviation from normal

patterns of defecation or alterations
in the frequency, size, consistency, or
ease of passage of stool.

Constipation

Alterations : frequency
size
consistency
ease of passage of stool
Adult : pass at least 3 stools per week
The first week of life reveal decline from more
than : 4 stool per day
4 year age
: 1 2 stool per day

Anorectal Continence and

Management of Constipation

Colonic motility
Defecations
Continence Gross-m.puborektalis
Fine : m.sphincter
Encopresis (soiling)

Abdominal distension

Remembered by using the letter Fsix

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
Ax: Normal passage of stool,
Px: Ballotement, shifting dullness, fluid
thrill and fluctuates

Abdominal
Distensions

Side view
Pubo-Xyphoid line:
small intestinal dilatations

Upper view
Angulus costa-SIAS:
Colon dilatations

Frog like abdomen

Abdominal distention
compartement mnemonic F6:
- Feses (Fecaloma,fecalith)
- Fetus in feto
- Fibroids (Solid tumor)
- Flatus (Ileus)
- Fluid (Ascites)
- Fat

Symptoms and Signs

of Surgical Disease
Vomiting

Bile-free vomitus is hallmark of gastric outlet obstruction in

the infant. It is not always due to Hypertrophyc Pyloric
Stenosis (HPS). HPS causes projectile nonbilious vomiting
usually starting at 2 to 6 weeks of age and failure to
thrive

Bilious vomiting on the first day of life, without abdominal

distentio, is the cardinal sign in patient with a duodenal
obstruction.

Early Intestinal
Pathologic

Obstipations
Distended abdomen
Vomiting

Caused Ileus

Colicy abdominal pain simple ileus

Strangulated pain
- strangulated ileus

The cardinal symptom of

Intestinal obstruction (Ileus)

Obstipation
(absolute constipation)
Distension
Colic
Vomiting

Definition ileus
Classification ileus:
1. Manifestations Clinic
- Upper
- Lower : - Mechanic
- Functional
2. Radiologic (Post Barium meal,enema)
- Upper (forgut)
- Intermediate (midgut)
- Lower (hindgut)

HIGH GIT OBSTRUCTION

GASTRIC OUTLET OBSTRUCTION
- HPS ( HYPERTROPHIC PYLORIC STENOSIS )*
- ANTHRAL WEB*
- PYLORIC MUCOSA PROLAPS*
DUODENAL OBSTRUCTION
- ATRESIA/STENOSIS DUODENUM*
- PANCREAS ANNULARE*
- LADD`S MEMBRANE*
*) all requiring surgery

MECHANICAL LOWER GIT

OBSTRUCTION

MECONIUM ILEUS*
MECONIUM PLUG SYNDROME*
NEONATAL SMALL LEFT COLON SYNDROME*
MALROTATION WITH VOLVULUS*
INCARCERATED HERNIA*
JEJUNOILEAL ATRESIA*
COLONIC ATRESIA*
INTESTINAL DUPLICATION*
INTUSSUSCEPTION*
HERNIA INGUINALIS*

*) all requiring surgery

FUNCTIONAL LOWER GIT

OBSTRUCTION

SEPSIS
NEC (NECROTICANS ENTERO COLITIS)*
INTRACRANIAL HEMORRHAGE*
HYPOTHYROIDISM
MATERNAL DRUG INGESTION OR ADDICTION
HYPERMAGNESEMIA
HYPOKALEMIA
MORBUS HIRSCHSPRUNG*

*) requiring surgery

Age, and the common cause of

alimentary tract obstruction

Birth Atresia (duodenum,ileum)

- Meconium ileus
- Volvulus neonatorum
3 weeks- Congenial Hypertrophyc Pyloric Stenosis
6 9 month Intussusception
Teen-age Inflammatory mass (appendicitis)
- Intussusception of Meckels diverticulum
or polyp
Young adult Hernia and adhesion
Adult Hernia, adhesions, inflammation (appendicitis,Crohn
disease), Carcinoma
Elderly- Carcinoma, Inflammation (diverticilitis)
sigmoid volvulus

Symptoms and Signs

of Surgical Disease

Abdominal pain : visceral and somatic pain

The features in the history of pain that must be elicited
1.History: Trauma - caused from solid + hollow organs
Non trauma caused from hollow organs
2.Site
3. Time and mode of onset
4.Severity
5.Nature (burning,throbbing,stabbing,constricting,
colicky,aching)

Symptoms and Signs

of Surgical Disease

1a
2

HOLLOW ORGANS: Locations of

abdominal pain
1.INTESTINUM:a.FOREGUT,
b.MIDGUT,c.HIND GUT
2. DUCTUS BILIARIS
3. DUCTUS PANCREATICUS
4. URINARY TRACT
5. TUBA FALLOPII

1b
4

4
5

5
4

1c 4

-Gall bladder pain goes through to the back

and to the right, to reach the tip of the shoulder
- Stomach and duodenum pain straignt
through to the back
-Pancreatic pain tends to go through to the
back but to the left

Causes
Abdominal pain

NONORGANIC: EMOSINAL, CENTRAL

NERVUS DISORDER, ORGAN
INTRABDOMINAL NORMAL
ORGANIC: DISORDERS ORGAN INTRA
ABDOMINAL AND ABDOMINAL WALL

ABDOMINAL PAIN-MANIFEST
DISORDERS ORGANIC
INTRAPERITONEUM
A.VISCERAL PAIN:
REFERRED PAIN NERVUS TH X
1. COLICKY/ CRAMPING PAIN SIMPLE OBSTRUCTION)
INTERMITTEN PAIN
2. CONSTANS PAIN- ISCHEMIC
TISSUE
B.SOMATIC PAIN
IRITATIVE PERITONEAL PAIN
( DEFANS MUSCULER)

OBSTRUCTION

DEFINITION: SYNDROME DISTURB PASSAGE

HOLLOW VISCUS ORGAN
HOLLOW VISCUS ORGAN:
1. INTESTINUM
2. DUCTUS BILIARIS
3. DUCTUS PANCREATICUS
4. URINARY TRACT
5. TUBA FALLOPII

APPENDICITIS

The appendiceal rupture rate

remains high among children
and range from 30% to 70%
( Ponsky TA et al., JAMA Oct.
27,2004)
An appendix ruptured can lead to
peritonitis and abscess, that is
often delayed and misdiagnose
of appendicitis acute
Objective: To investigate the
association between appendiceal
appendicolith as the risk of
appendiceal abscess-peritonitis

Moving pain periumbilical area (visceral pain)

to Mac Burney pin point (somatic pain)

FREQUENCY

Incidence appendicitis :
USA : 1.1 / 1000 people per year
Indonesia : unknown data
Predispositions : Appendicolith may made both
diet low in fiber or hyperplasia submucosal
lymphoid and Peyeri patchs to respons a
bacterial or viral infection

PATHOLOGY
Appendiceal obstruction

Secreting mucosa

Secretion retained
Increase pressure

No infection

mucocele

infection

Atrophic mucosa

ball valve
Relief of
obstruction

Edema
Hyperemia
Exudate

Complication:
Gangrene
perforation

Peritonitis
Sepsis
shock

abscess

USG
(ultrasound examination)

Appendic: appendicolith
thick wall diameter 15,2 mm
(normal < 6mm)

APPENDICOGRAM

APPENDICITIS
5 GRADE: CLOUDS
CLASSIFICATIONS
I SIMPLE
OBSTRUCTION
II SUPPURATIVE
III GANGREN
IV RUPTURE
V ABSCES

CLINICAL APPENDICITIS
Invasive
Bacterial Viral

Lymphoid
Follicle
Hyperplasia

Partial
Obstructions
(Anorexia, 74-78%,
Nausea 61-92%)
Colicky Pain 80%
(Periumbilical)
N Th X

GALT-MALT,
Peyeri patch

Feces
Retensions
Intralumen
appendix

Low Residu Diet

IMMUNOCOMPROMISE

APPENDICOLITH

Total
Obstructions
(Vomiting 50%)

Ischemia
(Vascular
Compromise)

Constan Pain
Mac Burney point

Rupture
Peritonitis Absces

Everything will be simple after

systematized
Medical problem
Surgical proble Elective
- Emergencies
Anamnesis: Chief complain
History
Physical Examinations:
Laboratory: blood, urine etc.
X rays plain
contras
USG (non invasive)
CTscan MRI
Diagnosis
Treatment
Prognosis

TERIMAKASIH