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.