Incidence of Childhood Cancer

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 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 to 1993 N= 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 times greater risk of SMN than peers % within first 20 years.