Incidence of Childhood Cancer

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Incidence of Childhood Cancer
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• What is cancer ?
• Uncontrolled growth of cells
• Are these cancer cells abnormal?
• No, but their behaviour is.

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Combination Chemotherapy

• Use of two or more drugs administered together,
  which usually act at different phases of cell
  cycle and therefore kill more cells.

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What is a clinical trial?

• A standardised approach to the treatment of a
  certain disease which is treated the same in all
  participating centres.

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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.

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Treatment of Cancer

• Surgery
• Chemotherapy
• Radiotherapy

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Childhood Leukaemia

• Acute Lymphoblastic (ALL) 70
• Acute Myeloid Leukaemia (AML) 20
• Acute Undifferentiated (AUL) lt5
• Chronic Myeloid (CML) and Juvenile Chronic
  Myeloid (JCML) 5

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Acute Leukaemia

• 30 childhood cancers
• 4/100,000 children lt15years
• Peak incidence 1-5 years

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ALL - Clinical Features

• Fever
• Lymphadenopathy
• Hepatosplenomegaly
• Bleeding
• Bone pain

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ALL - Differential Diagnosis

• Non malignant -
• Infectious mononucleosis
• ITP
• Aplastic anaemia
• Malignant-
• Neuroblastoma
• Bone tumours

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Lymphoma

• 80 childhood lymphomas are NHL
• Almost all high grade
• Tendency to BM and CNS involvement
• Disease free survival 70-85

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Hodgkins Lymphoma

• Lower incidence than NHL
• Rare in childrenlt 10yrs
• Usually present with cervical adenopathy
• Often localised disease
• Disease free survival good
• Late effects considerable

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Brain Tumours

• Infratentorial -
• disturbance of gait and co-ordination
• cranial nerve palsy
• headaches and vomiting

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Brain Tumours

• Supratentorial -
• Headaches
• Convulsions
• UMN signs
• Visual disturbance

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Brain Tumours

• Post fossa commonest site
• Gliomas gtPNETgtependymoma gtothers
• Surgery offers best chance of cure
• Some tumours chemosensitive
• Most tumours radiosensitive but avoid if possible
  lt4yrs.
• Overall survival 50 approx

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Differential Diagnosis of Malignant Abdominal
Tumours

• Neuroblastoma
• Wilms tumour
• Non Hodgkins lymphoma
• Soft tissue sarcoma
• Hepatoblastoma

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Neuroblastoma

• Commonest extracranial tumour
• Tumour of neuroectodermal origin
• Incidence 7-8/million lt 15 years
• Peak incidence 2-5 years

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Neuroblastoma - Clinical Features

• Depends on local, regional and metastatic spread
• Metabolic effects
• Greatest mimicker in paediatric practice

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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
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Wilms Tumour

• Arises from the kidney
• Incidence 7/million lt15 years
• Peak incidence 2-5 year age group

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Wilms Tumour - Clinical Features

• Asymptomatic abdominal mass
• Abdominal discomfort
• Haematuria
• Hypertension

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Rhabdomyosarcoma

• Tumour of mesenchymal origin
• Commonest STS in childhood
• Incidence 5-6 of childhood cancers

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Rhabdomyosarcoma - Clinical Features

• Occurs in all sites - 35 head neck
• Prognosis depends on primary site -
• paratesticular gt90, head neck 30
• peripheral - worst prognosis, usually
  alveolar.
• Histology major prognostic indicator

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Bone Tumours

• Comprise 5 of childhood cancers.
• Unusual lt5years of age
• Ewing osteosarcoma commonest
• Up to 20 will have metastases at diagnosis.

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Ewing Sarcoma- Clinical Features

• Pain usually gt6 months
• Palpable mass
• Pathological fracture
• Fever

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Osteosarcoma - Clinical Features

• Pain usually weeks cf months
• Commonest around knee
• Commoner in adoloscence
• Up to 20 metastases at presentation.

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Bone Tumours - Differential Diagnosis

• Ewing sarcoma
• Osteosarcoma
• Non Hodgkins lymphoma
• Langerhan cell histiocytosis
• Aneurysmal bone cyst
• Acute osteomyelitis

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Hepatoblastoma

• Presents most commonly 1-3yrs
• Large mass R hypochondrium
• aFP usually grossly elevated
• Usually chemosensitive
• DFS gt80
• Liver transplant rarely indicated.

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Germ cell tumours

• 40 sacrococcygeal
• May arise in gonads
• Usually chemosensitive
• AFP sensitive indicator

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Oncology Survival Curve Patients Diagnosed 1983
to 2003
70
59
1983 to 1993 N 729 1993 to 2003 N 1,160
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Late Effects of Childhood Cancer

• Depend on
• Disease
• Age
• Treatment

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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.