Paediatrics - Oncology Flashcards
What is Leukaemia?
What are the 3 types of leukemia that affect children?
What does leukemia do to the full blood count?
Give 5 risk factors for leukaemia
Leukaemia is the name for cancer of a particular line of the stem cells in the bone marrow. This causes unregulated production of certain types of blood cells. Types of leukaemia can be classified depending on how rapidly they progress (chronic is slow and acute is fast) and the cell line that is affected (myeloid or lymphoid).
The types of leukaemia that affect children from most to least common are:
Acute lymphoblastic leukaemia (ALL) is the most common in children - peak age is 2-3 years
Acute myeloid leukaemia (AML) is the next most common - peak age is under 2.
Chronic myeloid leukaemia (CML) is rare
Leukaemia is a form of cancer of the cells in the bone marrow. A genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell.
The excessive production of a single type of cell can lead to suppression of the other cell lines, causing underproduction of other cell types. This results in a pancytopenia, which is a combination of low:
Red blood cells (anaemia),
White blood cells (leukopenia)
Platelets (thrombocytopenia)
Risk factors:
- radiation exposure - X-ray during pregnancy
- Down’s syndrome
- Kleinfelter syndrome
- Noonan syndrome
- Fanconi’s anaemia
A 3 year old presents with:
Persistent fatigue
Unexplained fever
Failure to thrive
Weight loss
Night sweats
Pallor
Petechiae and abnormal bruising
Unexplained bleeding
Abdominal pain
Generalised lymphadenopathy
Unexplained or persistent bone or joint pain
Hepatosplenomegaly
Petichiae or hepatomegaly requires urgent referal for assessmenet.
What is the likely diagosis?
Investigations to establish the diagnosis?
Further investigations?
Management?
Leukemia
Ix:
- FBC - within 48 hours - shows anaemia, leukopenia, thrombocytopani and high numbers of abnormal white cells
- Blood film - blast cells
- Bone marrow biopsy
- Lymph Node biopsy
Further tests may be required for staging:
Chest xray
CT scan
Lumbar puncture
Genetic analysis and immunophenotyping of the abnormal cells
Management:
Leukaemia is primarily treated with chemotherapy is the main treatment
HPSC transplant for relapse or high risk disease
Prognosis - cure rate is 80% for ALL, lower for AML. Chemo can cause impaired development, immunodeficiency and neurotoxicity.
Whilst leukemias are the most common type of childhood cancer, brain cancer is also very common and is the leading cause of childhood cancer death.
How do brian tumours present?
5 types of paediatric brain tumours?
1 Key Ix
Treatment - general
Presentation:
- progressive focal neurological symptoms depending on the location of the lesion (e.g. a change in behavoir indicates the frontal lobe; peripheral weakness; bladder or bowel issues)
- Sx of intercranial hypertension - costant headache that is nocturnal/worse on waking, worse on coughing or bending forward and causes vomitting or papilloedema on fundoscopy
From lecture:
- headache
- vomitting
- papiodema
- squint
- nystagmus
- ataxia
- personility change
Note - some block the forth ventricle and cause hydrocephalus with Headache, naseau and vomitting (but this can just be caused by raised ICP and mass effects anyway)
Types:
These are almost always primary in children and are the leading cause of childhood cancer deaths in the UK.
1. Astrocytoma - varies from benign to highly malignant (glioblastoma). Pilcocytic astrocytoma is common benign astrocytoma seen in children.
2. Medulloblastoma - most common primary malignant brain cancer in children. Arises in cerbellum’s primative neuorepithelial cells.
3. Ependymoma - mostly in the posterior fossa where it behaves like medulloblastoma (ependyma is the ventrical lining). A type of glial cell tumour
4. Brainstem glioma
5. Craniopharyngioma - developmental tumour arising from an embryological remnant.
6. pinealoma - from the pineal region
Investigations
MRI scan is the first-line investigation in patients with a possible brain tumour.
Biopsy gives the definitive histological diagnosis, usually obtained during surgery to remove the tumour.
Management
- Surgery - also VP shunt to treat hydrocephalus
- Chemo
- Radio
What is neuroblastoma?
Presentation?
Investigations?
Management?
A tumour of the neural crest cells which normally develop into the cells of sympathetic chain and the adrenal medulla (most common site of tumour).
Neuroblastoma most common cancer in infants. Rarely seen above the age of 5.
Presentation:
- fever, weightloss, sweating and fatigue (from chemokines released by the tumour) Basically if spread to the bone marrow it makes them feel shit.
- other symtoms are due to the location of the tumour - spine (muscle weakness),
- adrenal medullar - large painful adominal mass
- metastic bone pain and fractures (base of the skull
- thoracic- breahting difficulties, neck -> horner syndrome
- pancytopenia if spread ot bone marrow
Ix:
- Raised urinary catecholamine levels
- CT scan
- Biopsy
- Bone marrow biopsy to check for metastic disease in the bone marrow
Rx:
- Surgery can often be curative when there is no metastatic disease
- Metastatic disease will need chemotherapy, surgery and radiotherapy
A 3 year old presents with signs and symptoms of:
Abdominal pain
Haematuria
Lethargy
Fever
Hypertension
Weight loss
- Most likely diagnosis?
- Investigation - inital and definative
- Management?
Wilms’ tumour is a specific type of tumour affecting the kidney in children, typically under the age of 5 years.
Diagnosis
The initial investigation is an ultrasound of the abdomen to visualise the kidneys. A CT or MRI scan can be used to stage the tumour. Biopsy to identify the histology is required to make a definitive diagnosis.
Management
Treatment involves surgical excision of the tumour along with the affected kidney (nephrectomy).
Adjuvant treatment refers to treatment that is given after the initial management with surgery. This depends on the stage of the disease, the histology and whether it has spread. The main options are:
Adjuvant chemotherapy
Adjuvant radiotherapy
Prognosis
Early stage tumours with favourable histology hold a good chance of cure (up to 90%). Metastatic disease has a poorer prognosis.
A teenager presents with persistant pain in their thigh, that wakes them from sleep.
What 2 inital investigations are required? What do they show?
Likely diagnosis?
Management?
Osteosarcoma is a type of bone cancer. This usually presents in adolescents and younger adults aged 10 – 20 years. The most common bone to be affected is the femur. Other common sites are the tibia and humerus.
Ewing sarcoma – a form of bone and soft tissue cancer most often affecting children and young adults. This is more common in younger children. May also show onion skin appearence
The main presenting feature is persistent bone pain, particularly worse at night time. This may disturb or wake them from sleep.
Other symptoms that may be present include bone swelling, a palpable mass and restricted joint movements.
Diagnosis:
NICE guidelines recommend a very urgent direct access xray within 48 hours for children presenting with unexplained bone pain or swelling. If the xray suggests a possible sarcoma they need very urgent specialist assessment within 48 hours.
Xrays show a poorly defined lesion in the bone, with destruction of the normal bone and a “fluffy” appearance. There will be a periosteal reaction (irritation of the lining of the bone) that is classically described as a “sun-burst” appearance. There can an associated soft tissue mass.
Blood tests may show a raised alkaline phosphatase (ALP).
Further investigations is used to better define the lesion and stage the cancer:
CT scan
MRI scan
Bone scan
PET scan
Bone biopsy
Management involves surgical resection of the lesion, often with a limb amputation. :( :( :( - v sadAdjuvant chemotherapy is used alongside surgery to improve outcomes.
Begin tumours - osteomas are managed with observation.
What is retinoblastoma? Bilateral vs unilateral?
Three most common signs and symptoms of retinobalstoma?
Ix?
Rx?
Malignant tumour of retinal cells, which accounts for around 5% of visual impairment in children and can be unilateral or bilateral
Bilateral tumours are hereditary, unilateral tumours are caused by a mutation that occours later in development.
Retinoblastoma susceptibility is on chromosome 13 with dominant inheritance
Most cases present in the first 3 years of life
Generally prognosis is very good.
Presentation
- White pupillary reflex replaces the red one
- Squint/strabismus
- visual impairement
It is unlikely to be painful
Ix:
- opthalmoscopy
- MRI of the eye
Rx:
- cryotherapy
- laser therapy
- surgery
- chemo (is metastic)
Aim to cure but preserve vision.
A small card on hepatoblastoma:
What is hepotoblastoma?
How does it present?
key initial Ix?
Management?
This is a malignant (cancerous) tumour of the liver which usually occurs in young children. This is different from hepatocellular carcinoma, which usually occurs in adults and occasionally in older children. The blastomas are the paediatric ones- retinoblastoma, neuroblastoma…
rare - 10 children a year in the UK
Usually presents with abdominal distension or with a mass, pain and jaundice is usually rare
- Elevated a-fetoprotein is detected in nearly all cases of hepatoblastoma.
- Management includes chemotherapy, surgery and in inoperable cases, liver transplantation is needed.
- Prognosis is generally good and children will be cured.
Differentiating leukemias based on bone marrow biopsy. What is seen for ALL, AML and CLL?
ALL most ocmmon (75% of paediatric cases), Lymphoid progenitor cells (lyphoblasts) in the bone marrow.
AML - blood film and bone marrow biopsy will show a high proportion of blast cells (myeloid). Auer rods in the cytoplasm of blast cells are a characteristic finding in AML.
CML is often associated with Philadelphia chromosome (t(9;22)).
