Malignant Diseases Flashcards
Define Wilm’s Tumour.
Wilms tumour originates from embryonal renal tissue and is the most common renal tumour of childhood.
When do Wilm’s tumours present?
Over 80% of patients present before 5 years of age and it is very rarely seen after 10 years of age.
How do Wilm’s tumours present?
What are the investigations of Wilm’s Tumours?
What is the management of Wilm’s tumours?
- In the UK, children receive initial chemotherapy
- Followed by delayed nephrectomy
- After which the tumour is staged histologically and subsequent treatment is planned according to the surgical and pathological findings.
- Radiotherapy is restricted to those with more advanced disease.
- Initial nephrectomy followed by chemotherapy is an approach taken in some countries.
- Around 5% of patients have bilateral disease at diagnosis, and their management is directed to preserve as much renal function as possible.
What is the prognosis of Wilm’s Tumours?
Prognosis is good, with more than 80% of all patients cured. Cure rate for patients with metastatic disease at presentation (~15%) is over 60%, but relapse carries a poor prognosis.
How to brain tumours differ in children to adults?
- In children, almost always primary rather than metastatic
- 60% are infratentorial (located below tentorium cerebelli)
- Most common solid tumour in children
- Leading cause of childhood cancer death in UK
What are the types of paediatric brain tumours? Briefly describe them.
o Astrocytoma (40%): varies from (low grade) to highly malignant (high grade, *glioblastoma multiforme* ). o Medulloblastoma (20%): arise in midline of the posterior fossa, 20% have spinal metastases at diagnosis o Ependymoma (8%): most common site is IVth ventricle but may arise anywhere in CSF spaces. 10% are slow growing low grade but the remainder present as aggressive tumours requiring complete resection and radiotherapy for cure. o Brainstemglioma(6%):poorprognosis o Craniopharyngioma (4%): developmental tumour arising from the squamous remnant of Rathke pouch. Not truly malignant but locally invasive and grows slowly in suprasellar region o Atypical teratoid/rhabdoid tumour
What do the clinical features of brain tumours depend on?
- Age
- Site of tumour
How do brain tumours present in all ages?
o Persistent or recurrent vomiting
o Problems with balance, coordination or walking
o Behavioural change
o Abnormal eye movements
o Seizures, without fever
o Abnormal head position: wry neck, head tilt or persistent stiff neck
How do brain tumours present in children/adolescents?
o Persistent or recurrent headache
o Blurred or double vision
o Lethargy
o Deteriorating school performance
o Delayed or arrested puberty, slow growth
How do brain tumours present in infants?
o Developmental delay
o Progressive increase in head circumference, separation of sutures, bulging fontanella
o Lethargy
How do brain tumours from different sites present?
o Supratentorial(cortex)
▪ Seizures
▪ Hemiplegia
▪ Focal neurological signs
o Midline
▪ Visual field loss: bitemporal hemianopia
▪ Pituitary failure: growth failure, diabetes insipidus, weight gain
o Cerebellar and 4th ventricle
▪ Truncal ataxia
▪ Coordination difficulties
▪ Abnormal eye movements
o Brainstem
▪ Cranial nerve defects
▪ Pyramidal tract signs
▪ Cerebrallar signs: ataxia
▪ Often no raised ICP
• Spinal tumours can present with back pain, peripheral weakness of arms or legs or bladder/bowel dysfunction
What are the investigations for brain tumours?
- Brain tumours are best characterized on MRI scan.
- Magnetic resonance spectroscopy can be used to examine the biological activity of a tumour and aid radiological diagnosis.
- Some tumour types can metastasize within the CSF and a lumbar puncture is therefore required for complete staging of the disease.
- Lumbar puncture must not be performed without neurosurgical advice if there is any suspicion of raised intracranial pressure.
14 yr old
Aggressive behaviour at school, headaches, seizures
10 yr old complaining of headaches, nausea, poor growth, struggling to see board at school.
3 yr old vomiting in the morning, unsteady on his feet, new onset convergent squint.
4 yr old. Refuses to walk, unable to climb, squint, facial asymmetry and drooling.
How should we manage brain tumours?
- Surgery is usually the first treatment and is aimed at:
- treating hydrocephalus
- providing a tissue diagnosis
- attempting maximum resection.
- In some cases the anatomical position of the tumour means biopsy is not safe,
- e.g. tumours in the brainstem and optic pathway.
- Even tumours which are histologically ‘benign’ can threaten survival.
- The use of radiotherapy and/or chemotherapy varies with tumour type and the age of the patient.
In the acute phase, neurorehabilitation including physiotherapy, occupational therapy, speech and language therapy may be required to support optimal recovery. Survivors living with disability, growth, endocrine, neuropsychological, and educational problems require complex care management into adulthood.
How common is leukaemia in children?
Leukaemia most common type of cancer in childhood in developed world
What are the types of of leukaemia?
ALL accounts for 80% of leukaemia in children.
o Others: acute myeloid leukaemia, acute non-lymphocytic leukaemia
o CML and other myeloproliferative disorders are rare
What are the clinical features of ALL?
Peak age of ALL presentation is 2-5 years
Symptoms occur due to disseminated disease and systemic illness from infiltration of bone marrow or other organs with leukaemic blast cells
Presents insidiously over several weeks usually
General: malaise, anorexia
Bone marrow infiltration
o Bonepain
o Anaemia → pallor, lethargy
o Neutropenia → infection
o Thrombocytopenia → bruising, petechiae, nose bleeds
• Reticulo-endothelial infiltration
o Hepatosplenomegaly
o Lymphadenopathy (superior mediastinal obstruction) – uncommon
• Other organ infiltration
o CNS→headaches, vomiting, nerve palsies
o Testes → testicular enlargement
What are the investigations for ALL?
• FBC will be abnormal
o Usually with a low Hb, thrombocytopaenia and evidence of circulating leukaemic blast cells
Bone marrow biopsy - essential to confirm diagnosis
Clotting screen
o 10% of patients with acute leukaemia have DIC at the time of diagnosis
Lumbar puncture can reveal disease in the CSF
CXR can reveal a mediastinal mass (characteristic of T-cell disease)
How are ALL and AML classified?
Both ALL and AML are classified by morphology. Immunological phenotyping further subclassifies ALL; the common B-cell (75%) and T-cell (15%) subtypes are the most frequent. Prognosis and some aspects of clinical presentation vary according to different subtypes, and treatment intensity is adjusted accordingly.
What factors contribute to prognosis of ALL?
