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Incidence of Childhood Cancer
Leukaemia
Brain tumours
20%
32%
Lymphoma
10%
Rhabdomyosarcoma
Neuroblastoma
Wilms' tumour
8%
5% 2%
5%
5%
6%
7%
Bone tumours
Retinoblastoma
Germ cell tumours
Others
• What is cancer ?
Uncontrolled growth of cells
• Are these cancer cells abnormal?
No, but their behaviour is.
Combination Chemotherapy
• Use of two or more drugs administered
together, which usually act at different
phases of cell cycle and therefore kill more
cells.
What is a clinical trial?
• A standardised approach to the treatment of
a certain disease which is treated the same
in all participating centres.
Why the need for clinical trials?
• So that the best approach to treatment is
arrived at as quickly as possible by treating
the largest number of patients in the shortest
possible time.
Treatment of Cancer
• Surgery
• Chemotherapy
• Radiotherapy
Childhood Leukaemia
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Acute Lymphoblastic (ALL) 70%
Acute Myeloid Leukaemia (AML) 20%
Acute Undifferentiated (AUL) <5%
Chronic Myeloid (CML) and Juvenile
Chronic Myeloid (JCML) 5%
Acute Leukaemia
• 30% childhood cancers
• 4/100,000 children <15years
• Peak incidence 1-5 years
ALL - Clinical Features
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Fever
Lymphadenopathy
Hepatosplenomegaly
Bleeding
Bone pain
ALL - Differential Diagnosis
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Non malignant Infectious mononucleosis
ITP
Aplastic anaemia
MalignantNeuroblastoma
Bone tumours
Lymphoma
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80% childhood lymphomas are NHL
Almost all high grade
Tendency to BM and CNS involvement
Disease free survival 70-85%
Hodgkins Lymphoma
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Lower incidence than NHL
Rare in children< 10yrs
Usually present with cervical adenopathy
Often localised disease
Disease free survival good
Late effects considerable
Brain Tumours
• Infratentorial disturbance of gait and co-ordination
cranial nerve palsy
headaches and vomiting
Brain Tumours
• Supratentorial Headaches
Convulsions
UMN signs
Visual disturbance
Brain Tumours
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Post fossa commonest site
Gliomas >PNET>ependymoma >others
Surgery offers best chance of cure
Some tumours chemosensitive
Most tumours radiosensitive but avoid if
possible <4yrs.
• Overall survival 50% approx
Differential Diagnosis of
Malignant Abdominal Tumours
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Neuroblastoma
Wilms’ tumour
Non Hodgkins lymphoma
Soft tissue sarcoma
Hepatoblastoma
Neuroblastoma
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Commonest extracranial tumour
Tumour of neuroectodermal origin
Incidence 7-8/million < 15 years
Peak incidence 2-5 years
Neuroblastoma - Clinical
Features
• Depends on local, regional and metastatic
spread
• Metabolic effects
• Greatest mimicker in paediatric practice
NBL – Survival Curve
Patients Diagnosed 1993 to 2003
Kaplan Meier 1993 to 2003
100
90
80
(76%)
70
Stage
60
1
2
3
4
4S
50
40
(30%)
30
Stage 1
N= 3
Stage 2
N = 12
10
Stage 3
N = 9
0
Stage 4
N = 34
20
0
2
4
6
Time
8
10
12
Stage 4S N = 6
Wilms’ Tumour
• Arises from the kidney
• Incidence 7/million <15 years
• Peak incidence 2-5 year age group
Wilms’ Tumour - Clinical
Features
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Asymptomatic abdominal mass
Abdominal discomfort
Haematuria
Hypertension
Rhabdomyosarcoma
• Tumour of mesenchymal origin
• Commonest STS in childhood
• Incidence 5-6% of childhood cancers
Rhabdomyosarcoma - Clinical
Features
• Occurs in all sites - 35% head &neck
• Prognosis depends on primary site paratesticular >90%, head & neck 30%
peripheral - worst prognosis, usually
alveolar.
Histology major prognostic indicator
Bone Tumours
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Comprise 5% of childhood cancers.
Unusual <5years of age
Ewing & osteosarcoma commonest
Up to 20% will have metastases at
diagnosis.
Ewing Sarcoma- Clinical
Features
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Pain usually >6 months
Palpable mass
Pathological fracture
Fever
Osteosarcoma - Clinical Features
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Pain usually weeks cf months
Commonest around knee
Commoner in adoloscence
Up to 20% metastases at presentation.
Bone Tumours - Differential
Diagnosis
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Ewing sarcoma
Osteosarcoma
Non Hodgkins lymphoma
Langerhan cell histiocytosis
Aneurysmal bone cyst
Acute osteomyelitis
Hepatoblastoma
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Presents most commonly 1-3yrs
Large mass R hypochondrium
αFP usually grossly elevated
Usually chemosensitive
DFS >80%
Liver transplant rarely indicated.
Germ cell tumours
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40% sacrococcygeal
May arise in gonads
Usually chemosensitive
AFP sensitive indicator
Oncology Survival Curve
Patients Diagnosed 1983 to 2003
Kaplan Meier 1983 to 2003
100
90
80
70%
70
59%
60
Years
50
1983 to 1993
1993 to 2003
40
30
20
10
0
0
10
20
Time
30
1983 to 1993
N= 729
1993 to 2003
N = 1,160
Late Effects of Childhood Cancer
Depend on:
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Disease
Age
Treatment
SMN
• Depends on - primary cancer
- treatment
- genetic predisposition
- age at diagnosis.
• Adult survivors of childhood cancer 10-20
times greater risk of SMN than peers.
• 12-20% within first 20 years.