Pediatric Solid Tumors Kuliah S1

Pediatric Solid Tumors
Bambang Sudarmanto
Hematology-oncology working group
Kariadi Hospital Semarang
Indonesia
11/02/15 06:34
bambangsdmt@gmail.com
11/02/15 06:34
Pediatric Tumors
Kariadi
Hospital is referall hospital Central

Java and part of Borneo
Class of patients: Jamkesmas (poor
people), Askes (health insurance), private
Many are treated in Private Hospitals
many
hospitals do not treat children with

cancer
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Pediatric Solid Tumors (<14)

9,000
new cases, 1500 deaths of pediatric

cancers annually in the U.S.
Cancer- 2nd leading cause of death in
children (1st infections)
1/3 deaths from leukemia
CNS tumors most frequent SOLID tumors
in pediatric population
Indonesia data not viable (Semarang?)
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Pediatric solid tumors

Childhood
requires
cancer
multidisciplinary treatment groups
emotional
needs of patient/family
nutritional needs
coordinated care- medical, surgical, oncology,
nursing, social services, therapists, educators
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Distribution of Childhood Cancer

NBL
8%
Soft Tissue
Sarcoma
7%
Wilm's
Tumor
6%
Bone
5%
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Hodgkin's
NHL
5%
3%
Germ Cell
Other
3%
8%
CNS
18%
Liver
1%
Leukemia
33%
Retina
3%
6
Pediatric Neck Mass
Congenital
Thyroglossal
Duct Cyst
Acquired
Branchial
cleft cyst
Infectious/
Inflammatory
Neoplasms
Cystic
hygroma
Dermoid
cyst
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Congenital Masses
Thyroglossal
duct
Laryngoceles
cysts
Branchial cleft cysts
Lymphatic
malformations
Hemangiomas
Teratomas
Dermoid cysts
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Thymic
cysts
Vascular
malformations
SCM tumors of
infancy
Plunging ranulas
Acquired Masses
Infectious
Inflammatory
Bacterial
Kawasaki
Viral
Sinus
Fungal
Sialadenitis
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Histiocytosis
Drug-induced
lymphadenopathy
Sarcoidosis
Abdominal masses
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10
Abdominal masses
History
Symptoms
abdominal
discomfort, increased abdominal

size or assymptomatic
Presence of systemic symptoms
bone
Other
pain, limping, malaise, fever
symptoms
hematuria
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11
Abdominal masses
Physical findings
Presence of abdominal mass
upper
Other
abdomen or lower abdomen
associated physical findings
other
masses: orbital
bruises
hypertension
pain
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12
Abdominal Mass
Differential diagnosis
depends on location
Upper abdominal
mass
Neuroblastoma
Wilms tumor
Hepatoblastoma
Rhabdomyosarcoma
Germ
cell tumor
Lymphoma
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13
Abdominal Mass
Differential diagnosis (cont)
Lower abdominal/pelvic
mass
Neuroblastoma
Rhabdomyosarcoma
Germ
cell tumor
Lymphoma
Ewings sarcoma
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14
Abdominal Masses
Laboratory evaluation
Bone marrow aspirate
neuroblastoma,
lymphoma,
rhabdomyosarcoma or
Ewings sarcoma
CSF
tap
if
lymphoma is a
consideration
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15
Abdominal masses
Radiologic work-up
Abdominal/pelvic ultrasound
useful
screening test: helps define location and

quality (solid or cystic)
not useful to assess for retroperitoneal adenopathy
Computed
tomography
location
of mass
presence of calcification
obstructive signs
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16
Wilms Tumor
Accounts
for 6% childhood malignancies

Median age at diagnosis 3 years
Metastasizes to lung and lymph nodes
70%
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patients present with localized disease
17
Wilms Tumor: Signs & Symptoms

Most
commonly
presents as painless
abdominal mass
Hypertension (2030%)
Pain (20-30%)
Hematuria (25%)
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18
Wilms Tumor
Diagnosis:
Ultrasound
CT
Lymph
node and lung mets

Right sided tumors- liver mets
Mets to bone and brain possible with some
histologies
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19
Wilms Tumor: Staging System

Stage
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Definition
Tumor limited to kidney, completely excised
II
Tumor extends beyond the kidney, completely

resected; no residual tumor beyond resection margins
III
Residual non-hematogenous tumor confined to

abdomen
IV
Hematogenous metastases
Bilateral renal involvement
20
Bilateral Wilms
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21
Survival by Stage and Histology
Histology/stage
# pts
2 yr-s*
4 yr-s*
FH/I
546
98
97
II
281
96
94
III
290
91
88
IV
126
88
82
UH/I
20
89
89
UH/II-IV
40
56
54
survival
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22
Wilms tumor
Treatment:
Remove
kidney- nephrectomy
Lymph
nodes biopsied
Can spread also through blood
Tumor spill is possible- abdominal cavity is at risk
Chemo
and RT- sometimes preop to reduce

tumor spill
RT late
stages, residual dx, LN mets,

bilateral dx, lung mets
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23
Wilms Tumor
Chemo
Actinomycin-D
Added
vincristine, Adriamycin- 80-100% cure rates

Mets = 70% cure rate
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24
Wilms Tumor: Prognosis & Future Directions

Prognostic
Future
factors: stage and histology
directions
Minimize
therapy for favorable histology
patients
Identify biologic factors predictive of outcome
Intensify therapy for patients with unfavorable
histology
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25
Pediatric Brain Tumors

(CNS Tumor)
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26
Brain tumors: pediatric vs. adult

Adults:
70% of tumors are supratentorial
meningioma
pituitary
adenoma
High grade astrocytoma
Anaplastic
astrocytoma (grade III)

Glioblastoma multiforme (grade IV astrocytoma)
Pediatric:
70% in posterior fossa
pilocytic
astrocytoma (cerebellar astrocytoma)

medulloblastoma
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27
CNS TUMORS
Most
common solid tumors in children

2nd most frequent (16.6% of all childhood
malignancies)
Incidence has increased over the past 2
decades
Leading cause of cancer death in children
Result in more morbidity than any other
malignancy in childhood
Metastatic brain cases are rare
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28
CNS TUMORS
Signs
& Symptoms
(related to the location, histologic grade
of tumor & age of child)
General
-Headache
-Seizures
-Mental status changes
-Increased intracranial Pressure (ICP)
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29
Astrocytoma
- high grade
- low grade
Medulloblastoma
Benign tumor : adenoma
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30
Medulloblastomas
25% of all childhood brain tumors

Occurs in the posterior fossa
2-12 yr old
Arises in the midline of the cerebellum and spreads to the in the

4th ventricle and brain stem
High propensity to spread through CSF
peaks at 5 yr old
Rare in adults
arises from primitive neuroepithelial cells
histologically cells appear as small, round, blue cells
forming pseudorosettes. Purple friable tumor
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31
medulloblastomas
invasion
of the fourth ventricle causes sx
CSF
does not flow causing hydrocephalus

headaches early- morning vomit get progressively
worse
Ataxia
cranial nerve abnormalities from invasion of the
brain stem
Staging correlates with survival
CT/MRI, myelography of entire CNS necessary
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32
Medulloblastomas
Can
develop mets in bone

Neurosurgery is first treatment
remove
gross disease
ventricular shunts are discouraged because of possible
seeding into peritoneal cavity
CSI-
Craniospinal Irradiation
postop-
RT
lateral- opposed brain fields
cover
retroorbitally through mid-cervical cord

The divergence of the spinal field determines the collimator
twist
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33
CSI-
Craniospinal Irradiation
Depending
on the length of the spine, two spinal fields

may be necessary
Whole spine is treated- down to S2 or S3
Electrons are used many times to avoid treating
anterior structures
Gaps (photons) between fields are feathered
Some institutions are encouraging a small gap
between lateral brain fields and the upper spinal field
See page 326 Red
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34
CSI -Craniospinal Irradiation

Immobilization
important
What
position?
Anesthesia may be necessary
RT Doses:
1.5
Gy day to 36 Gy
Boost to posterior fossa area- 54 Gy @ 1.8
day
Chemo
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investigation
35
Retinoblastomas
Most
common intraocular tumor in

children
200 U.S. annually (Indonesia: no data)
Most occur 6 mos-4yrs old
Hereditary risk factors- 10% of cases
Can
be bilateral- 35% cases
Rarely
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curative
36
Retinoblastomas
Discovered
White
by light reflex
vs. red
Ophthalmologist
No
biopsy
Avoid
possibility of vitreous seeding
CT: detect
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exam
extraocular extension
37
Retinoblastomas
CSF
disease
Assessed
by lumbar puncture
Bone
marrow may be biopsed

Staging systems developed
Preferred
treatments outlined
Predict success rates
Most common
Reese-Ellsworth
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38
Retinoblastomas
Treatment Techniques
Photocoagulation
Cryosurgery
Small
tumors not near optic disc and macula
Enucleation
Unilateral
disease
Sacrifices globe
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39
Retinoblastomas
Treatment Techniques:
Surgery
Radiation
Implants
RT
Inoperable
or bilateral dx
Spare lens and cornea
Treat retina
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40
Retinoblastomas
Treatment
Method: if disseminated dx
Chemo
Good
response expected
VAC- Vincristine, Adriamycin, cyclophosphamide
OPEC- vincristine, cisplatin, etoposide,
cyclophosphamide
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41
Retinoblastomas
Late
Effects of Treatment
Enucleation
Several
prosthetics will be needed as child grows
RT
Facial
growth affected
Small orbits
Blind spots
Radiation retinitis, and dry eyes
2nd malignancy
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42
Neuroblastomas
500
cases annually U.S.

2nd most common pediatric solid tumorafter brain tumors
Age range- newborns-several yrs old
Median age 2 yrs old
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43
Neuroblastomas
Occur
mainly in the abdomen
Originate
in adrenal gland or paraspinal ganglia
Lethargic,
ill appearing child under 2 with

abdominal mass is common
Spinal cord invasion will promote neurological sx
Horners syndrome present with upper
sympathetic ganglia involvement
Flushing, diarrhea
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44
Neuroblastomas
Many
children present with mets

Children older than 18 months have 70%
chance of developing mets
CT- chest, abd (US)
Lungs
and liver assessed
MRI
spine
Bone marrow biopsy
Bone scan
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45
Neuroblastomas
Prognosis
Dependent
on age at presentation
< 1 yr old disease may regress if acute sx are
managed
Treatment
Techniques
RT and
chemo- low cure

Surgery if no mets- high cure rate
Chemo and BMT
RT- palliation
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46
Lymphoma
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47
Lymphomas
3rd
most common cancer in children. 60% are

NHL; 40% Hodgkins disease
Extranodal disease is common in children with
1/3 rd involving the mediastinum, abdomen or
head and neck
Multi agent chemotherapy is the mainstay of
treatment, surgery and radiotherapy
Anticipation and treatment of complications is
extremely important (lysis tumor syndrome, SVC
syndrome)
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48
Non Hodgkins lymphoma

NHL result
from malignant proliferation of

cells of lymphocytic ar histiocytic linage.
Generally restricted to lymphoid tissue
such as lymphnodes and spleen
NHL categorized as low, intermediate and
high grade on the base of their clinical
aggressiveness.
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49

Epidemiology
peak is between 5-15 years median age 9

years; male preponderance , 3:1
Increase risk include patient with immunologic
defect (AIDS, etc), immunosuppresive therapy
(post transplant)
EBV is almost invariably associated with the
African Burkitts lymphoma
11/02/15 06:34
50

Clinical
feature:
are
varied depend on the location of the tumor, the

extent of the disease (stage) and histologic subtype
Extranodal involvement :
Mediastinum: may produce SVC syndrome
Abdomen: ileocaecal region, appendix, colon and
retroperitoneal area. Cayse pain, vomiting,
intussuception, ascites, obstructive
Head and neck: enlargement of cervical node, paritis,
tonsilar hyperthropy; jaw swelling
11/02/15 06:34
51

Diagnosis
and staging:
History
and physical exam

Blood count, BMA and Biopsy
LDH level
Evaluation of liver test (bil, SGOT/SGPT;
alkphospatase)
Chest radiography; USG; CT Scan abdomen
Adequate surgical biopsy
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52

Staging:
Stage
I: a single tumor (extranodal) or single

anatomic areawith exclusion of mediastinum
and abdomen
Stage II: stage I + two or more nodal area on
the same side of diaphragna
Satege III: stage II + all extensive node
enlargement
Stage IV: stage III + with initial involvement of
CNS or bone marrow or both
11/02/15 06:34
53

Principal
Multi
therapy :
agent chemotherapy : CHOP
Anticipation
Two
and treatment of complication:
complication :
- Arising Space occupying tumor:
intrathoracic complication
-
pericardial effusion
Metabolic complication of chemotherapy:
tumor lysis syndrome
Management of relaps
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54
Bone tumors
Benign
and malignant tumor

Fracture pathologic
Plain radiography is lytic bone lesion;
cortical destructions soft tissue extension.
Two most common cancers of bone :
osteosarcoma and Ewing sarcoma and
the fascinating group of disorders known
as histiocytosis
11/02/15 06:34
55
Osteosarcoma
Epidemioloy
and etiology:
Most
frequent adolescent and young adults

Annual 600 new cases in USA
Correlated with radiation exposure
Incidence increase among survival
retinoblastoma (Rb) and p53 tumor supressor
gen.
11/02/15 06:34
56
Osteosarcoma
Pathology:
Clinical
history and radiographic appereance

of bone lesion
Correct diagnosis requires of bone biopsy
Biology
Genetic
alteration occur in the tumor

suppresor genes p53 and Rb to play
important role in tumorogenesis
11/02/15 06:34
57
Osteosarcoma
Pathology:
Clinical
history and radiographic appereance

of bone lesion
Correct diagnosis requires of bone biopsy
Biology
Genetic
alteration occur in the tumor

suppresor genes p53 and Rb to play
important role in tumorogenesis
11/02/15 06:34
58
Osteosarcoma
Clinical
presentation:
Painful
hard of the bone

History of trauma
Osteosarcoma can occur in any body but usually
develop in metaphysis of long bone, common site is
distal femur; proximal humerus,
Systemic symptom are fever, night sweats, weight
loss.
Metastatic disease: other bone; pulmonary; soft tissue
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59
Osteosarcoma
Diagnosis
and staging:
Plain
radiography lytic and/or sclerotic bone

abnormality
A sunburst appearance
Cortical distruction and periosteal reaction
MRI and biopsy
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60
Osteosarcoma
Treatment
: local control of tumor and systemic

therapy to eradicated microscopic metastasis
disease at distant sites
Preoperative
chemotherapy : reduced tumor bulk;

prognostic implication; making surgical resection
easier
HDX MTX; doxorubicin and cisplatin
Post operative chemotherapy 12 month
Non metastatic disease : 60 -65% to be cured
Metastatic
11/02/15 06:34
diseases indicated poor prognosis

61
Ewing sarcoma
Ewing
sarcoma is one of a group of

tumors referred to as the Ewing sarcoma
family tumors (ESFT): Ewing sarcoma of
bone; soft tissue Ewing sarcoma and
peripheral primitive neuro ectodermal
tumor (PNET)
Arise from share the same chromossomal
abnormality
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62
Ewing sarcoma
Epidemiology
and etiology
Less
than 20 years at diagnosis

Annual incidence 2.7 case permillion
The cause of ESFT is unknown, unrelated
with radiation
Pathology
11/02/15 06:34
and biology:
63
Soft Tissue Sarcomas

Arise
from mesenchymal tissue

Can arise anywhere in body
Wide variety of histologies
Most
common 60 %
Rhabdomyosarcomas
Undifferentiated
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64

Diagnosis
and Staging
Depends
on area involved
H&N, genitourinary, extremities and trunk
A large
painless mass- extremity

Painful orbit
In
genitourinary areas- urinary problems

(male)
Exophytic mass vagina
Mets to lung and bone marrow most common
11/02/15 06:34
65

Staging
Physical
exam
US
CT or
MRI
BM biopsy/Chest CT (mets)
Stage and histology dictate tx
11/02/15 06:34
66
Staging
Treatment
Surgery
First
choice is removal
RT
Improves
local control, spare organs

Field covers entire muscular compartment
50.4 Gy 1.8 Gy fx
Shield growth plates
Spare BM in pelvis if possible
Brachy
Chemo-
resected/nonresected tumors
Vincristine
11/02/15 06:34
and actinomycin-D
67
Unusual Childhood Tumors

Germ
Cell Tumors
Can
occur anywhere in the midline from CNS to

ovaries and testes
Sacrococcygeal teratomas often seen in newborns
Nonseminomas
Embryonal
carcinoma
Choriocarcinoma
Yolk-sac tumors
These often produce AFP, HCG chemical markers
Seminoma-
11/02/15 06:34
dysgerminoma
68

Liver
tumors
Appear much like Wilms tumor or
neuroblastoma
CT or US will differentiate
Hepatoblastoma-
under 2 yrs old

Hepatocellular carcinoma- after 10 yrs old, multifocal
Surgery- primary tx
Chemo-
often used
RT- rarely used
Poor survival rates
11/02/15 06:34
69

Histiocytosis
X syndromes
Spectrum
of disease
Involves Langerhans cells
Miscellaneous Tumors
Nasopharyngeal
angiofibromas
Undifferentiated nasopharyngeal
Thyroid tumors- benign and malignant, Iodine 131ablation
Keloids or fibromas
Kaposis sarcoma
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70
Pediatric Patients- Special

Considerations
Extra
Time Needed
Anesthesia
Psychosocial
needs of family- jobs/home
Understanding/compassionate
therapist
Use
of rewards- stickers/toys
Protocols
Info
sent for immediate review

Careful adherence to guidelines important
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71
Thank for your attention

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72

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Pediatric Solid Tumors

Hospital is referall hospital Central

hospitals do not treat children with

Pediatric Solid Tumors (<14)

new cases, 1500 deaths of pediatric

Pediatric solid tumors

multidisciplinary treatment groups

Distribution of Childhood Cancer

Pediatric Neck Mass

discomfort, increased abdominal

pain, limping, malaise, fever

abdomen or lower abdomen

associated physical findings

screening test: helps define location and

for 6% childhood malignancies

patients present with localized disease

Wilms Tumor: Signs & Symptoms

node and lung mets

Wilms Tumor: Staging System

Tumor limited to kidney, completely excised

Tumor extends beyond the kidney, completely

Residual non-hematogenous tumor confined to

Bilateral renal involvement

Survival by Stage and Histology

and RT- sometimes preop to reduce

stages, residual dx, LN mets,

vincristine, Adriamycin- 80-100% cure rates

Wilms Tumor: Prognosis & Future Directions

factors: stage and histology

therapy for favorable histology

Pediatric Brain Tumors

Brain tumors: pediatric vs. adult

70% of tumors are supratentorial

astrocytoma (grade III)

70% in posterior fossa

astrocytoma (cerebellar astrocytoma)

common solid tumors in children

25% of all childhood brain tumors

Arises in the midline of the cerebellum and spreads to the in the

of the fourth ventricle causes sx

does not flow causing hydrocephalus

develop mets in bone

retroorbitally through mid-cervical cord

on the length of the spine, two spinal fields

CSI -Craniospinal Irradiation

common intraocular tumor in

be bilateral- 35% cases

possibility of vitreous seeding

marrow may be biopsed

tumors not near optic disc and macula

prosthetics will be needed as child grows

cases annually U.S.

mainly in the abdomen

in adrenal gland or paraspinal ganglia

ill appearing child under 2 with

children present with mets

and liver assessed

chemo- low cure

most common cancer in children. 60% are

Non Hodgkins lymphoma

from malignant proliferation of

Non Hodgkins lymphoma

peak is between 5-15 years median age 9

Non Hodgkins lymphoma