Malignant disease Flashcards
What age do leukaemia, neuroblastoma, Wilms tumour, Hodgkin lymphoma and bone tumours present?
Leukaemia - children of all ages
Neuroblastoma and Wilms tumour - first 6 years of life
Hodgkin lymphoma and bone tumours - peak incidence in adolescence and early adult life
In children, which malignancy is the most common?
Leukaemia
Are there any inherited cancers?
Bilateral retinoblastoma.
What are some associations with disease and cancers?
Down syndrome and leukaemia
Neurofibromatosis and glioma
How can children with a cancer present?
A localised mass
The consequences of disseminated disease (bone marrow infiltration, systemic ill health)
The consequences of pressure from a mass on local structures or tissue (airway obstruction secondary to enlarged lymph nodes in the mediastinum)
What is increased urinary catecholamine excretion useful for confirmation?
Neuroblastoma
What is high Alpha fetoprotein production usually present in?
Germ cell tumours and liver tumours
Do children and infants or teenagers and young adults have a worse malignancy prognosis?
Teenagers and young adults due to their specific types and biological behaviour of their tumours and to their particular social/psychological needs
What are the different uses of chemotherapy in childhood malignancies?
A primary curative treatment (ALL)
Control primary or metastatic disease before definitive local treatment with surgery/radiotherapy (sarcoma/neuroblastoma)
Adjuvant treatment to deal with residual disease and to eliminate presumed metastases (Wilms tumour)
How is radiotherapy use different in children to adults?
The risk of damage to growth and function of normal tissue is greater in children.
The need for protection of normal tissues and for careful positioning and immobilisation of the patient during treatment poses practical issues
What is the role of bone marrow transplant with high-dose therapy?
The limitation of both chemo and radiotherapy is the risk of irreversible bone marrow damage, higher doses of these can be used if transplantation of bone marrow stem cells is given at the same time
What are the sources that can be used to give bone marrow stem cells?
Allogenic (from a compatible donor)
Autologous (from the patient themselves, harvester beforehand, while the marrow is uninvolved or in remission)
When are allogenic bone marrow stem cells used?
Principally, in the management of high-risk or relapsed leukaemia
When are autologous bone marrow stem cells used?
Most commonly in the treatment of children with solid tumours whose prognosis is poor using conventional chemotherapy (advanced neuroblastoma)
What are some of the side effects of chemotherapy?
Bone marrow suspension - anaemia, thrombocytopaenia and bleeding, neutropenia Immunosuppression - infection Gut mucosal damage - undernutrition Anorexia Nausea and vomiting Alopecia Potential infertility
What are some important infections to look out for in chemotherapy?
Pneumonia
Disseminated fungal infection
Coagulase-negative staph infections of central venous catheters
What psychosocial support is available for families with childhood malignancy?
Patient counselling regarding anxiety, guilt, fear and sadness.
Help with practical issues - transport, finances, accommodation and care of siblings
Detailed written material for both parents, siblings and patients
What is the most common cancer in children?
ALL followed by AML/ANLL.
How does ALL present and at what age?
Peaks at 2-5 years. Presents with disseminated disease and systemic ill-heath from infiltration of bone marrow or other organs with leukaemia blast cells. Most present insidiously over several weeks
What would a FBC usually show in ALL?
Low Hb
Thrombocytopaenia
Evidence of circulating leukaemic blast cells
What investigations would you perform in ALL?
FBC
Bone marrow examination
CXR
What are you looking for on a CXR in ALL?
A mediastinal mass characteristic of T-cell disease
What are the stages of treatment for high-risk ALL?
Induction Consolidation and CNS protection Interim maintenance Delayed intensification Continuing maintenance
What does the induction stage of ALL treatment involve?
Combination chemotherapy is given and current induction treatment schedules achieve remission rates of 95%