Incidence of Childhood Cancer

10%

8%7%6%5%5%2%5%

20%32%

Leukaemia

Brain tumours

Lymphoma

Rhabdomyosarcoma

Neuroblastoma

Wilms' tumour

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

• 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

Su

rv

iva

l P

rob

ab

ilit

y (

%)

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

Su

rv

iva

l P

rob

ab

ilit

y (

%)

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.