CANCER IN CHILDREN
By: Cecilia Galan- Fernandez, DPPS, DPSHBT, DPSPO
Place: 5th floor ASMPH
Date : January 11,2011
All kinds of cancer, including
childhood cancer, have a
common disease process
cells grow out of control,
•develop abnormal sizes and
shapes
•ignore their typical boundaries
inside the body
• destroy their neighbor cells
•spread (or metastasize) to other
organs & tissues
• As cancer cells grow, they demand
more nutrition from the body
•
Normally, your body forms new cells
as you need them, replacing old
cells that die
Cell cycle
•Incidence
14/100,000
•Among
all age groups, the most
common childhood cancers are leukemia,
lymphoma, and brain cancer.
•
As children enter their teen years, there is an increase
in the incidence of osteosarcoma (bone cancer).
•The
sites of cancer are different for each type
Pediatric vs. Adult Cancers
Lifestyle factors that contribute to the
development of cancer in adults:





smoking
diet
obesity
occupation
prolonged exposure to carcinogenic
agents
For the 0-14 yrs old
Acute leukemias (ALL-most common)
Central nervous system tumors
Neuroblastoma
Wilms’ tumor
Non Hodgkin’s Lymphoma
Rhabdomyosarcoma, soft tissue tumors,germ cell
tumors, retinoblastoma and bone tumors
For 15 – 19 yrs old
Hodgkin’s disease
Germ cell tumors
Osteosarcoma, Ewing’s sarcoma
Pediatric vs. Adult Cancers
Lifestyle factors are not associated with
cancer in children
Genetic
condition- anomalies
Family history
Environmental- ionizing radiation, chemicals
Infections – EBV
Chemotherapy
Precise cause – unknown
Signs of Childhood Cancer
Continued, unexplained weight loss
Headaches, often with early morning vomiting
Increased swelling or persistent pain in bones, joints, back, or legs
Lump or mass, esp. in the abdomen, neck, chest, pelvis, or armpits
Development of excessive bruising, bleeding, or rash
Constant/recurrent infections
A whitish color behind the pupil
Nausea which persists or vomiting with or w/o seizure
Constant tiredness or noticeable paleness
Eye or vision changes which occur suddenly and persists
Recurrent or persistent fevers of unknown origin
WHY DIFFICULT TO DIAGNOSE
CHILDHOOD CANCER?

Vague manifestations

Childhood malignancies are rare

Doctor’s reluctance to consider cancer
diagnosis
Important Factors to Cure

EARLY DETECTION

PROPER TREATMENT

GOOD SUPPORTIVE CARE
RETINOBLASTOMA
RETINOBLASTOMA
Most common intraocular malignancy of
childhood
 2nd most freq solid malignancy
 80% are diagnosed before 3-4 years old
 Median age at diagnosis: 2 years old
 Beyond 6 years old is rare!!!
 Incidence:
1. 60% --non-heriditary & unilateral
2. 25% --hereditary & bilateral
3. 15% --hereditary & unilateral
CLASSIFICATION
1.Laterality ---unilateral or bilateral
2.Focality ---Unifocal or multifocal
3.Genetics --- hereditary or
nonheriditary
LATERALITY
UNILATERAL
-Knudson’s two hit
phenomenon
-15% carry
constitutional
mutation

BILATERAL
-asstd. with advanced
parental age
-present earlier than
unilateral
-hereditary form
-worse prognosis
-autosomal dominant

Retinoblastoma




RB gene on Chromosome 13, “two-hit theory”
Round blue cell tumor arising in any of the
nucleated layers of the retina
Hematogenous, lymphatic spread, direct extension
thru the optic nerve
Only 10% detected by ophthalmologic screening
COMMON SIGNS &
SYMPTOMS
1.
2.
3.
4.
5.
6.
7.
Leukokoria --most common
Strabismus --2nd most common
Decreased visual acuity
Inflammatory changes
Glaucoma
Vitreous hemorrhage resulting in black pupil
Rubeosis iridis –(neovascularization of surface
of iris)
*in 50% with advanced dsespontaneous bleed--hyphema
Investigations for Diagnosis of Retinoblastoma
1.Examinations
-done under anesthesia by pedia ophtha
-done by pedia oncologist
-Audiology evaluation if chemo with carbplatin
2. Imaging studies
-CT scan of brain and orbits
3. Laboratory evaluations
-CBC,Bld chem,electrolytes
-Creatinine clearance if chemo with carboplatin
Investigations for Diagnosis of Retinoblastoma
4. Diagnostic Studies
-Lumbar puncture ---only if with radiographic or
clinical suspicion of CNS disease
-Bone scan --- only if with bone pain or other extraocular disease
-Bone marrow biopsy –only with abnormal
CBC(without alternative explanation) or other
extraocular disease
5.Pathologic evaluation---if enucleation is performed
Histology: The only human tumor that is
radically treated without tissue biopsy
confirmation
Flexner-Wintersteiner rosettes: lined by tall cuboidal cells that
circumbscribe and an apical lumen
Patterns of Spread
Intraocular:
1. With endophytic growth, there is a white hazy
mass.
2. With exophytic growth, there is retinal
detachment.
3. Most tumors have combined growth.
4. Retinal cells frequently break off from the main
mass and seed the vitreous or new locations on the
retina.
Extraocular:
1.
Retinoblastoma spreads first to surrounding structures and then by
hematogenous or lymphatic extension.
2.
Retinoblastoma invades the optic nerve. From there it can spread
directly along the axons to the brain or may cross into the
subarachnoid space and spread via the CSF to the brain.
3.
Hematogenous spread leads to metastatic disease, most commonly
to brain, bone marrow, or bone.
4.
Lymphatic spread is rare because there is minimal lymphatic
drainage of the orbit. Occasionally, retinoblastoma spreads
lymphatically to the preauricular and submandibular nodes.
PARTS OF THE EYE
retinoblastoma
SITES OF METASTASIS
1.
2.
3.
4.
BRAIN
BONE
BONE MARROW
LUNGS
RISKS FOR METASTATIC SPREAD
1. If optic nerve stump is <5mm—bad
>5mm---better
2. Tumor invasion into the anterior chamber
3. Large tumor with vitreous seeding
4. Rubeosis iridis
5. Glaucoma
Retinoblastoma
GOAL OF TREATMENT:
Primary Goal: Cure!!!!
Secondary Goal: Preservation of vision
TREATMENT
I.
Surgery
Enucleation—if no hope for salvage of useful vision
II. Radiotherapy
III. Chemotherapy
Neuroblastoma

Most common extracranial
solid tumor in infancy

8-10% of all childhood
malignancies

Affects infants & preschool
– 50% younger than 2
year old
– 30% younger than 1
year old
NEONATAL NEUROBLASTOMA

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Subcutaneous nodules. If massaged, a zone of pallor develops
around the nodule due to catecholamine discharge
Extensive metastasis to the liver
Bone marrow involvement
Massive adrenal hemorrhage into tumor
Hydrops fetalis and/or signs of erythroblastosis fetalis
Stage 4S
Diagnosis: prenatal ULTZ
In many cases, a mass is not palpable
Only 50% have elevated urinary catecholamines
Prognosis: favorable course (if with + favorable factors)
Treatment: Curative by surgery
Course:
Others regress
NEUROBLASTOMA



Originates from primordial neural crest cells that
normally give rise to adrenal & sympathetic ganglia
Are tumors of the sympathetic nerve tissue
Can occur anywhere along the sympathetic neural
pathway
Neuroblastoma – Clinical Features


Arise from the sympathetic outflow
Primary sites
– 70% abdominal primary
(50% adrenal medulla, 50% extra-adrenal tissue)
– Thoracic tumours, posterior mediastinum (20%)
– Head & neck tumours (10%)
– Epidural (dumbbell or hourglass shaped) tumors – cord
compression/paraplegia
– Bone
– Lymph nodes are enlarged
– Lungs—rarely involved(0.7%), involvement should be
proven by bx
– Pelvis---constipation,urinary retention,presacral
MANIFESTIONS
1.



Excessive catecholamine (VMA/HVA)
intermittent attacks of sweating,pallor
Headaches,palpitation
Hypertension—renin induced due to renovascular
compromise & seen in 1-5%
MANIFESTATIONS
2. Signs & symptoms of Vasoactive Intestinal
Peptide (VIP)



Intractable watery diarrhea resulting to failure to
thrive
Abdominal distention
hypokalemia
MANIFESTATIONS
3. Acute myoclonic encephalopathy
 Bursts of rapid involuntary random eye movements in all
directions of gaze (OPSOCLONUS)
 Motor incoordination due to frequent,irregular jerking of
muscles of the limbs and trunk (MYOCLONIC JERKING)
Note:
1.May or may not resolve after the tumor is removed or
symptoms may resolve only after several months. Some , it
may be permanent
2. Prognosis is good
PATHOPHYSIOLOGY OF OM




NOT DUE due to direct involvement of the CNS by tumor or
due to the production of catecholamines
It is associated by well-defined IgG & IgM autoantibodies that
bind to the cytoplasm of cerebellar Purkinje cells & to some
axons in the white matter
Diffuse & extensive lymphocytic infiltration with lymphoid
follicles is a characteristic histologic feature of OM
This suggests an IMMUNE-MEDIATED MECHANISM OF THIS
RARE SYNDROME
Recommended Criteria at INSS
Conference for a Diagnosis
of Neuroblastoma



OR


Established if:
(1) Unequivocal pathologic diagnosisa is made from tumor tissue by
light microscopy
(with or without immunohistology, electron microscopy), and/or
increased urine or
serum catecholamines or metabolites.b
(2) Bone marrow aspirate or trephine biopsy contains unequivocal
tumor cellsa (e.g.,syncytia or immunocytologically positive clumps of
cells) and increased urine or
serum catecholamines or metabolites
Source: Manual of Pediatric Hematology & Oncology (4th ed) p534
Recommended Studies for Assessment
of Extent of Disease
Tumor site
Primary site
Metastatic sites
Recommended tests
CT and/or MRI scana with 3-D measurements; MIBG scan.b
Bone marrow
Bilateral posterior iliac crest marrow aspirates and
trephine core bone marrow biopsies required to exclude
marrow involvement.
A single positive site documents marrow involvement. Core
biopsies must contain at least 1 cm of marrow (excluding
cartilage) to be considered adequate.
Bone
MIBG scan; 99Tc scan required if MIBG scan negative or
unavailable. Plain radiographs of positive lesions.
Lymph nodes
Clinical examination (palpable nodes
confirmedhistologically.
CT scan for nonpalpable nodes (3-D measurements).
Abdomen/liver
CT and/or MRI scana with 3-D measurements.
Chest
AP and lateral chest radiographs. CT/MRI necessary if chest
radiograph positive, or if abdominal mass/nodes extend
into chest
NB – Clinical features

Metastases
– Bone marrow- anaemia
– Bone (Pain)
– Orbit (Peri-orbital
– Skin (blue-berry
muffin)
NB – Therapy



Surgery –laminectomy to
decompression
Chemotherapy-responsive in
80-85%;advantageous in making
surgery safer by reduction of
tumor size
Transplant (Stage 4, Nmyc
amplification
– HD Chemo with Autologous
Bone Marrow Transplant
Evaluation of a Lymph Node



A diagnosis of lymphoma or any malignancy must always
be considered when evaluating a child with an enlarged
peripheral lymph node
It must be emphasized that not all palpable nodes are
pathologically enlarged and that most pathologically
enlarged nodes are benign.
Points to consider in evaluating a lymph node:
I. LOCATION OF THE NODE
a. Nodes of the axilla, neck and groin are often palpable
in normal children
b. Palpable supraclavicular nodes -- always considered
abnormal
* if with left-sided (Virchow’s) – metastases from
intra-abdominal CA
* if with right-sided nodes --- intrathoracic disease
II When size matters

a.Cervical and axillary nodes --- > 1 cm

b. Inguinal node ------ >1.5 cm

c. Epitrochlear node ----> 0.5 cm
IV.INDICATIONS
III. GENERALIZED OR LOCALIZED?
a. Generalized --- involvement of more than two (2)
noncontiguous areas
caused by many different disease
process
b. Localized
---- enlarged lymph nodes within
contiguous anatomic regions and
usually due to one of two ( infection or
malignancy)
III. CHARACTER
Malignant nodes ---- non-tender, firm, rubbery and
rapidly enlarging becoming
cnonfluent and located in the
supraclavicular region
INDICATIONS FOR LYMPH NODE BIOPSY


Chronic,persistent,progressive adenopathy
If an infectious etiology has not been
uncovered, a dominant node that persists for
6 weeks should be biopsied
.



Node > 2.5 cm in diameter in the absence of
signs of infection that warrant a trial of
antibiotic
Supraclavicular adenopathy
Sytemic symptoms
Non-Hodgkin Lymphoma
–
–
–
–
–
–
–
5-7% malignant disease of childhood
3rd most common childhood malignancy
60% of childhood lymphomas
M>F
Peak age: 5-15 years old
Etiology: unknown
Risk factors:
 Genetic-- SCID, WAS
 Posttransplantation immunosuppression
 Drugs ex. Phenytoin (cause pseudolymphoma that
regress with cessation of treatment)
 Radiation – treatment with radiotherapy (4-5%
develop NHL within 10 years
 Virus---EBV and HIV
APPROACH TO THE DIAGNOSIS OF NON
HODGKIN’S LYMPHOMA
I.
History and PE
childhood lymphoma more often extranodal rapid tumor
growth
frequently involved site: Intra-abdominal ( B-cell)
Intrathoracic (precursor
T-cell)
Sites:
Abdomen --- primary site (35%)
Head and neck ---- 13%
Mediastinum ---- 26%
CNS and BM --- CNS involvement and epidural tumor
uncommon at presentation but more
common in the presence of BM
involvement.
--- 2/3 with BM disease have simultaneous
CNS involvement
--- Intracranial tumor more likely epidural
although it can occur within brain
parencyma
B SYMPTOMS OF HODGKIN
LYMPHOMA
1. Unexplained fever with temp above 38.0ْ C
orally
2. Drenching night sweats
3. Unexplained weight loss of 10% within 6
months preceding diagnosis
Source: Principles & Practice of Pediatric Oncology 5th ed p.701
IMPORTANT INVESTIGATIONS FOR NHL



Histologic analysis- Primary mode of
definitive diagnosis
CBC and APC
Chemistries and Serum electrolytes
– Liver and renal function tests
– SLDH- measures tumor volume
– Uric acid

Imaging studies
– Chest X-ray
– Chest CT
– Abdominal CT/ Ultrasound


BMA with biopsy ( bilateral)
CSF examination -- essential
ST. JUDE CHILDREN’S STAGING
SYSTEM
STAGE I

A single tumor (extranodal) or single anatomic area (nodal) with the exclusion of
mediastinum or abdomen
STAGE II



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A single tumor (extranodal) with regional node involvement
Two or more nodal areas on the same side of the diaphragm
Two single (extranodal) tumors with or without regional node involvement on the same
side of the diaghragm
A primary gastrointestinal tract tumor, usually in the ileocecalarea, with or without
involvement of associated mesenteric nodes only
STAGE III

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Two single tumors (extranodal) on opposite sides of the diaphragm
Two or more nodal areas above and below the diaphragm
All the primary intrathoracic tumors (mediastinal, pleural and thymic)
All extensive primary intraabdominal disease
All paraspinal or epidural tumors, regardless of other tumor site(s)
STAGE IV

Any of the above with initial CNS and/or bone marrow involvement
Lymphomas –
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Hodgkin Lymphoma
Childhood form < 14yo, young
adult form 15-34yo, older adult
form 55-74yo
EBV implicated, <10 yrs, male,
with preexisting
immunodeficiency
Reed-Sternberg cell hallmark
of disease
Painless lymphadenopathy,
anterior mediastinal mass and
systemic symptoms like fever,
night sweats and weight loss
Ann Arbor Staging System
Same + neck CT, ESR, serum
copper and ferritin
3rd most common

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Non- Hodgkin Lymphoma
Classified as B or non-B types
(previously classified as
lymphoblastic, SNCCL, LCL)
Intrathoracic, abdominal skin ,
bone, soft tissues, CNS
St Jude Staging system
CBC, electrolytes, uric acid,
sldh, creatinine, sgpt,chest
Xray or scan, abdominal scan
or MRI, bone scan, BMA or
biopsy, CSF cytology
WILMS’ TUMOR


Most common primary malignant tumor of
childhood
EPIDEMIOLOGY:
1975-1995 – 7.6 Cases per million
children
younger than 15 y/o
6% of childhood cancer
higher for blacks, low in Asians
F>M
A CHILD WITH ANIRIDIA
Wilms’ tumor associated syndrome (WAGR)
1. Wilms’ tumor
1.Aniridia
2.Hemihypertrophy
3.Genitourinary abnormalities
4. Mental retardation
Wilm’s Tumor
(Nephroblastoma)

Clinical Features:
1.Abdominal mass
-Synchronous (4.4-7%)
-Metachronous(1-1.9%)
2.Abdominal pain
3. Hematuria (10%)
4.Hypertension (25%)
PHYSICAL EXAMINATION


Location
Size of the mass
Note:
WT is usually a large flank mass that
does not particularly move with respiration.
WORKUP






CBC
Liver tests
Renal function tests (urinalysis,creatinine)
Serum calcium (elev.in rhabdoid or cmn)
Coagulation assays
Imaging studies:
CT (abdomen)
Doppler ULTZ –to assess the IVC
CXR/ CT (chest)
Bone scan or skeletal survey
Brain imaging (MRI)
STAGING (NWTS-5)


Stage I ---confined to the kidney &
completelyresected.
no renal capsule penetration
or involvement of renal sinus vessel
Stage II --- tumor extends beyond the kidney but
completely resected (neg.margins & LN)
At least 1 of the ff:
a. Penetration of the renal capsule
b. Invasion of renal sinus vessels
c. biopsy of tumor prior to removal
STAGING (NWHTS-5)


STAGE III --- Gross or microscopic residual tumor
remains postop,including
inoperable tumor
spillage of tumor preop or intraop
regional LN metastasis or
transected tumor thrombus
STAGE IV ----
Hematogenous or LN mets
outside the abdomen
(lung,liver,bone,brain)

STAGE V ----- Bilateral Wilms’ tumor
Predictive factors
(recurrence/progression
1.size
2.age of patient
3.histology
a .blastemal– hi stage/invasive
b. stromal
c. epithelial –low stage/ less
invasive and
resistant to
chemotx
PROGNOSIS


4. LN metastasis
5. local features of tumor – capsular or
vascular invasion
IMPORTANT:
The histologic finding of ANAPLASIA is the most important
determinant prognosis
BILATERAL WILMS’
TUMOR


Histology: favorable
Prognostic factors:
1. Age
diagnosis at early age (<2 y/o) –better
survival rate:
<2 y/o ---70-75%
>2 y/o --- 20-45%
2. Stage
survival rate:
Stage 1 & II ---85%
Stage III & IV --- 0%
POSITIVE PROGNOSTIC FACTORS
1.
2.
3.
4.
5.
6.
Stage I an II
Neg. Para-oartic nodes
Absence of anaplasia/sarcomatous
Absence of tumor rupture
Time of relapse (later better than early)
(> 15 months from diagnosis)
Site of metastases at relapse
(Lung better than liver)
NEPHROGENIC RESTS
TREATMENT

OBJECTIVE:
1. Maximum preservation of
renal tissue
2. Tumor removal by partial
nephrectomy
TREATMENT
1. Laparotomy and bx of both kidneys
(stage and histology)
LN should be bx & each side is staged
separately.
Excision of the tumor(s) is performed
at the initial operation only if all of
the tumor can be removed with
preservation of sufficient renal
parenchyma for normal renal
function on at least one side
2. Chemotherapy
Babies < 12 mo.– half the dose
Babies > 12 mo.– full dose
CHEMOTHERAPY

DOSE:
– A- Dactinomycin (45ug/kg,IV)
– D* Doxorubicin (1.0mg/kg, IV)
– D # Doxorubicin (1.5mg/kg,IV)
– V – Vincristine(0.05mg/kg,IV)
– V* Vincristine(0.67mg/kg,IV)
– XRT – Radiation therapy
** for StageIII and IV, FH or StageII-IV,UH
TREATMENT

Evaluation and Abdominal CT – at week 5
at wk 6 – if the imaging showed persistent
but resectable tumor or tumor
responded completely
do second look surgery and biopsy
• at 2nd look surgery ---complete
excision of the tumor frm.the least
involved kidney shld. be carried out.
• If the procedure leaves a viable &
functioning kidney on 1 side & the
other kidney, if extensively involved
with tumor, should be removed.
TREATMENT

Postoperative chemo shld. be adapted to
renal function abnormalities:
a.If 2nd look surgery showed no gross or
pathologic evidence of persistent tumor, chemo
be given accordingly
b. If 2nd look surgery showed gross or pathologic
evidence of persistence that can’t be resected,
do radiotx and change chemo
(ICE)
Abdominal Tumors
Neuroblastoma

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Peripheral sympathetic
nervous system malignancy
<5 yrs old, median 2 years
old, male predominance
Most common abdominal
malignancy
Shimada classification, MYC
gene
Retroperitoneal, palpable
causing abdominal discomfort,
opsomyoclonus, diarrhea
(+) calcification and
hemorrhage on CT
Bone, marrow liver, nodes skin
mets
Wilms’ tumor – 2nd

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
Nephroblastoma or mixed
embryonal neoplasm of the
kidney with three elements:
blastema, epithelia and stroma
2-5 years of age
WT1 gene (11p13) Denys-Drash
syndrome, WT2 gene BeckwithWiedenman syndrome, GUT
abn
Smooth and firm, pain, vomiting
and hematuria, hypertension
(-) calcification
Pulmonary metastasis, nodes
Abdominal Tumors



Neuroblastoma
CT or MRI, BMA, bone
scan, urine VMA and HVA
(95% +)
Prognostic factors: age at
diagnosis, stage, MYCN
status, Shimada histology
and ploidy in infants
Stage IV-S: <1year old,
with dissemination to the
skin, liver, bone marrow
without bone involvement
plus a primary tumor



Wilms’ tumor – 2nd
CT or MRI and chest
XRay
Prognosis affected by
stage and type of
histology, unfavoprable
in the presence of
large nuclei,
hyperchromatism,
multiple mitotic figures
Stage V – bilateral
tumors
HEPATOBLASTOMA
Liver Tumours



Hepatomegaly ± jaundice
Raised AFP (age-specific
normogram)
2 types:
– Hepatoblastoma
most common
 infants & young children

– Hepatocellular
carcinoma
Rare, older
 HBV, HCV

Rhabdomyosarcoma
Most common soft tissue sarcoma
 Found in any anatomic site: head/neck (40%), GUT
(20%), trunk (10%)
 Associated with Li Fraumeni syndrome and
Neurofibromatosis
 Arise from the same embryonic mesenchyme as
striated muscle
Types: embryonal (60%) intermediate prognosis
boytryoid, alveolar, pleomorphic,
undifferentiated

Clinical



Non-tender mass with no unusual hue to the
overlying skin or subcutaneous tissue
Growth of a nontender mass esp. without a clearcut
history of trauma, should always alert the MD to
consider biopsy, esp. if expansion is confirmed by
repeated observations over 1-2 weeks
Mass within a body cavity producing obstruction or
discharge,mandates a biopsy
Rhabdomyosarcoma



Diagnosis: disseminates early
CT/MRI, BMA, bone scans, lymph node biopsy
The most essential element of the work-up is
examination of the tumor tissue w/c include the use
of special stains(desmin, muscle specific actin,
MyoD).
Completely excised tumors have the best prognosis.
Favorable site: orbit
older children and disseminated diseases have
poorer prognosis
Rhabdomyosarcoma
 Sites
– Head & neck
 Orbit, nasopharyngeal
& middle ear tumours
– Genitourinary tract
 Bladder & prostate,
vaginal & uterine
– Extremity
 Prognosis
– Orbit - excellent
– GU tract - good
– Extremity, retroperitoneal,
metastatic - poor
Bone Tumours
Malignant Bone Tumors
Osteosarcoma







Most common primary
malignant bone tumors in
children
Second decade of life, growth
spurt, long bones, metaphysis
Pleomorphic, spindle shaped
cells with malignant osteoid
Bone pain and swelling
“Sunburst pattern” xray
Primary site: FEMUR
(metaphyseal portion)
Long bones
Ewing’s Sarcoma







More common in the first 10
years of life
Small round undifferentiated
tumors of neural crest
origin, flat bones, diaphysis
Askin tumor –if tumors arise
in the chest wall
S100 and NSE (+)
Pain and swelling, (+) fever
and weight loss
“onion-skinning” pattern
xray (flat & diaphyses)
Primary: pelvic,LE,bones of
chest wall
Radiological difference
Sunburst of Osteosarcoma
Onion skin of Ewings sarcoma
Malignant Bone tumors

Diagnosis: MRI or CT of tumor
SLDH
bone scan
Alkaline phosphatase
chest ct scan
bone biopsy/BMA in Ewing’s Sarcoma
Both with 75% cure rate if non-metastatic on
presentation.
Treatment: Multi-agent chemotherapy and surgery.
Radiotherapy also needed in Ewing’s
Sarcoma
Brain Tumours

Special problems
– Blood-brain barrier limits chemotherapy
– Developing brain is vulnerable to toxicity of
therapy
– Proximity of tumours to vital structures
precludes extensive surgery
– Tendency to spread within neuraxis
Brain Tumours Classification
Location
2/3 above &
1/3
below
tentorium
Site and symptoms

Posterior fossa – limited space, disrupt CSF
flow
– Vomiting, Increased ICP
– Motor tract involvement – cranial n, long tracts

Supratentorial
– Seizures – focal seizures
– Deterioration in school performance
– Hormonal defects – central precocity, diabetes
insipidus
(L) parieto-occipital low grade astrocytoma
Brain Tumours - Therapy

Surgery
– Resectability

Radiotherapy
– Toxicity




IQ drop
Endocrinopathy
Growth failure
Chemotherapy
– Prolong survival
– Increase cure rates
Prognosis
Prognosisdepends
dependson:
on
•Resectability
Resectability
•Age
Age
•Metastases
Metastases
•Chemosensitivity
Chemosensitivity
Oncologic emergencies
 Tumour
lysis syndrome
Tumour lysis syndrome


Metabolic derangement resulting from rapid death of
malignant cells
Must have 2 important factors :
– high tumour burden,
– highly sensitive tumour – ALL, AML, NHL

Hyperkalaemia - hydration, no K supplements, K binders

Hyperuricaemia – hydration, alkalinisation, allopurinol,
uricozyme to convert to allantoin

Hyperphosphataemia – Phosphate binders

Hypocalcaemia – if symptomatic, partial correction
Thank You!!!