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%
What is the consolidation and CNS protection stage of ALL treatment?
Cytotoxic drugs penetrate poorly into the CNS. As leukaemic cells in this site may survive effective systemic treatment, additional treatment with intrathecal chemotherapy is used to prevent CNS relapse
What is the maintenance therapy stage of ALL treatment?
Chemotherapy of modest intensity is continued over a relatively long period of time, up to 3 years from diagnosis
How would you treat a relapse of ALL?
High-dose chemotherapy, usually with total body irradiation (TBI) and bone marrow transplantation.
Are childhood brain tumours often primary or secondary?
60% are primary
What are the different types of brain tumours in children?
Astrocytoma (-40%, varies from benign to highly malignant) Medulloblastoma Ependymoma Brainstem glioma Craniophayngioma
Where does the medulloblastoma arise and where may it go?
Arises in the midline of the posterior fossa. May seed through the CNS via the CSF and up to 20% have spinal metastases at diagnosis
How does brain tumours often present?
Often related to evidence of raised ICP but focal neurological signs may be detected depending of the site of the tumour.
How would spinal tumours present?
Back pain, peripheral weakness of arms or legs or bladder/bowel dysfunction, depending on the level of the lesion
What is the best investigations for brain tumours?
MRI.
MR spectometry can be used to examine the biological activity of a tumour.
How would you manage a brain tumour?
Usually surgery, the use of radiotherapy and chemotherapy varies with the tumour type and the age of the patient
What are the late effect risks of brain tumour survivors?
Neurological disability of growth
Endocrine
Neuropsychological
Educational problems
What are lymphomas?
Malignancies of the cells of the immune system
Which lymphoma is more common in childhood?
NHL is more common in childhood, whereas Hodgkin lymphoma is seen more frequently in adolescence
What are the clinical features of Hodgkin lymphoma?
Painless lymphadenopathy, most frequently in the neck. Lymph nodes are much large and firmer than benign lymphadenopathy. Often long history (months) and systemic symptoms are uncommon
How would you investigate Hodgkin lymphoma?
Lymph node biopsy, radiological assessment of all nodal sites and bone marrow biopsy is used to stage disease and determine treatment. REED-STERNBERG CELLS
How would you treat Hodgkin Lymphoma?
Combination chemotherapy with to without radiotherapy. PET scan is used to monitor treatment response.
What is the prognosis of Hodgkin lymphoma?
80% of all patients can be cured. Even with disseminated disease, 60% can be cured
How can T-cell malignancies present?
As ALL or as NHL, both being characterised by a mediastinal mass with varying degrees of bone marrow infiltration.
How can B-cell malignancies present?
More commonly as NHL, with localised lymph node disease, usually in the head, neck or abdomen.
How would you investigate NHL?
Biopsy, radiological assessment of all nodal sites (CT or MRI) and examination of the bone marrow and CSF.
How would you treat NHL?
Multi-agent chemotherapy.
What is the prognosis of NHL?
Survival rates are over 80% for both T and B cell disease
Where does a neuroblastoma originate from?
It arises from neural crest tissue in the adrenal medulla and sympathetic nervous system.
What is unusual about neuroblastoma?
It can spontaneously regress in young infants
When is neuroblastoma most common?
Below the age of 5 years
What is the common presentation of neuroblastoma?
Pallor Weight loss Abdominal mass Hepatomegaly Bone pain Limp
What things are associated with a poor prognosis of neuroblastoma?
Overexpression of the N-myc oncogene, deletion of material on chromosome 1 and gain of genetic material on chromosome 17q in tumour cells
How would you treat neuroblastoma?
Localised primaries can be treated with surgery. Metastatic disease is treated with chemotherapy.
What is Wilms tumour?
It originates from embryonal renal tissue and is the commonest renal tumour of childhood.
When does Wilms tumour present?
Over 80% present before 5 years old, it is very rare over 10
How does Wilms tumour present?
Most children present with an abdominal mass, it is often found incidentally in an otherwise well child
What investigations would you do for Wilms tumour and what would you find?
US and/or MRI/CT. Usually characteristic intrinsic renal mass distorting the normal structure.
How would you treat Wilms tumour?
Initial chemotherapy followed by delayed nephrectomy. Prognosis is good, with more than 80% of patients being cured
What is the most common soft tissue sarcoma and where does it originate?
Rhabdomyosarcoma, originates from primitive mesenchymal tissue and there are a wide variety of primary sites, resulting in varying presentations and prognosis
When are bone tumours most common?
They are uncommon before puberty.
Osteogenic sarcoma and Ewing sarcoma are examples
What is retinoblastoma?
A malignant tumour of retinal cells.
It may affect one or both eyes
Can cause visual impairment
Bilateral tumours are hereditary
How are when does retinoblastoma present?
Most cases present before 3 years.
White pupillary reflex is noted to replace the normal red one or with a squint.
How do you treat retinoblastoma?
Chemotherapy
Local laser treatment
Radiotherapy
