incidence of childhood cancer
DESCRIPTION
Incidence of Childhood Cancer. What is cancer ? Uncontrolled growth of cells Are these cancer cells abnormal? No, but their behaviour is. Combination Chemotherapy. - PowerPoint PPT PresentationTRANSCRIPT
Incidence of Childhood Cancer
10%
8%7%6%5%5%2%5%
20%32%
Leukaemia
Brain tumours
LymphomaRhabdomyosarcoma
Neuroblastoma
Wilms' tumour
Bone tumours
RetinoblastomaGerm 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
• 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
• Fever• Lymphadenopathy• Hepatosplenomegaly• Bleeding• Bone pain
ALL - Differential Diagnosis
• Non malignant - • Infectious mononucleosis• ITP• Aplastic anaemia• Malignant-• Neuroblastoma• Bone tumours
Lymphoma
• 80% childhood lymphomas are NHL• Almost all high grade• Tendency to BM and CNS involvement• Disease free survival 70-85%
Hodgkins Lymphoma
• 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
• 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
• Neuroblastoma• Wilms’ tumour• Non Hodgkins lymphoma• Soft tissue sarcoma• Hepatoblastoma
Neuroblastoma
• 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
Stage12344S
Kaplan Meier 1993 to 2003
0 2 4 6 8 10 12
Time
100
90
80
70
60
50
40
30
20
10
0
Surv
ival
Pro
babi
lity
(%)
Stage 1 N = 3
Stage 2 N = 12
Stage 3 N = 9
Stage 4 N = 34
Stage 4S N = 6
(30%)
(76%)
Wilms’ Tumour
• Arises from the kidney• Incidence 7/million <15 years• Peak incidence 2-5 year age group
Wilms’ Tumour - Clinical Features
• 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
• Comprise 5% of childhood cancers.• Unusual <5years of age• Ewing & osteosarcoma commonest• Up to 20% will have metastases at
diagnosis.
Ewing Sarcoma- Clinical Features
• Pain usually >6 months • Palpable mass• Pathological fracture• Fever
Osteosarcoma - Clinical Features
• Pain usually weeks cf months• Commonest around knee• Commoner in adoloscence• Up to 20% metastases at presentation.
Bone Tumours - Differential Diagnosis
• Ewing sarcoma• Osteosarcoma• Non Hodgkins lymphoma• Langerhan cell histiocytosis• Aneurysmal bone cyst• Acute osteomyelitis
Hepatoblastoma
• Presents most commonly 1-3yrs• Large mass R hypochondrium• αFP usually grossly elevated• Usually chemosensitive• DFS >80%• Liver transplant rarely indicated.
Germ cell tumours
• 40% sacrococcygeal• May arise in gonads• Usually chemosensitive• AFP sensitive indicator
Oncology Survival Curve Patients Diagnosed 1983 to 2003
Years1983 to 19931993 to 2003
Kaplan Meier 1983 to 2003
0 10 20 30
Time
100
90
80
70
60
50
40
30
20
10
0
Surv
ival
Pro
babi
lity
(%)
1983 to 1993 N= 729
1993 to 2003 N = 1,160
59%
70%
Late Effects of Childhood Cancer
Depend on:
• 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.