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Incidence of Childhood Cancer. What is cancer ? Uncontrolled growth of cells Are these cancer cells abnormal? No, but their behaviour is. Combination Chemotherapy.
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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 (76%) (30%) Stage 1 N = 3 Stage 2 N = 12 Stage 3 N = 9 Stage 4 N = 34 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 • 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 70% 59% 1983 to 1993 N= 729 1993 to 2003 N = 1,160
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.