Haematology/Oncology Flashcards
What are the different types of tumours which are commonly seen in children?
- Acute leukaemia
- Brain/spinal cord
- Neuroblastoma
- Lymphoma
- Wilms tumour
- Bone tumour
- Retinoblastoma
How common is cancer in children?
1 in 600
What disease processes can pre-dispose to the development of cancer?
- Down
- Fanconi
- Beckwith Weiderman Syndrome
- Li-Fraumeni Familial Cancer Syndrome - p53
- Neurofibomatosis
What environmental factors can cause cancer?
-
Radiation
- Ionising
- Non-ionising - Sun
-
Viral infection
- EBV
- Hepatitis B
- HIV
-
Iatrogenic
- Radiotherapy
- Chemotherapy
What is the most common form of leukaemia in children?
Acute lymphoblastic leukaemia - 80%
What is the clinical presentation of ALL in children?
- Malaise/anorexia
- Pallor, lethargy - Anaemia
- Infection - neutropenia
- Bruising, petechiae, nose bleeds - thrombocytopenia
- Hepatosplenomegaly
- Lymphadenopathy - painless lumps
- Fever
- Headache, vomiting, nerve palsies - CNS involvement
How would you investigate if you suspected ALL?
- FBC - anaemia, variable WCC, decreased platelets
- Bone Marrow Biopsy - primarily blast cells
- Blood film - normochromic, normocytic anaemia
- CXR - mediastinal masses
- CSF - pleocytosis, increased protein, decreased glucose
What are the common sites for brain tumours in children?
- Cortex - astrocytoma
- Cerebellum - medulloblastoma, astrocytoma, ependymoma
- Brainstem - glioma
- Spinal cord - Astrocytoma, ependymoma
What are common presentations in children with brain tumours?
- Presistent/recurrent vomiting
- Balance, coordination, walking
- Behavioural change
- Abnormal eye movements
- Seizures
- Abnormal head position
What are symptoms of brain tumours which are specifically seen in children/adolescents?
- Persistent/recurrent headache
- Blurred/double vision
- Lethargy
- Delayed/arrested puberty
What are symptoms of brain tumours in infants?
- Developmental delay/regression
- Progressive increase in head circumference
- Bulging fontanelles
- Separation of sutures
- Lethargy
How would you investage a suspected brain tumour?
MRI scan
How does hodgkin’s lymphoma present in children?
- Painless lymphadenopathy - frequently around the neck
- B-symptoms - pruritis, fever, weight loss, sweating
What is a neuroblastoma?
A tumour that arises from neural crest tissue in the adrenal medulla and sympathetic nervous system. It can be benign (ganglioneuroma) to highly malignant (neuroblastoma).
What are common presenting symptoms of neuroblastoma?
- Pallor
- Weight loss
- Abdominal mass
- Hepatomegaly
- Bone pain
- Limp
Where is the most common site for neuroblastoma to occur?
Adrenal glands - leads to abdominal mass
What is a wilm’s tumour?
Nephroblastoma
A malignant tumor containing metanephric blastema, stromal and epithelial derivatives. Thought to be caused by mutation on the 11q13 chromosome
How does Wilm’s Tumour tend to present?
- Large abdominal/flank mass
- Haematuria
- Hypertension - renin secretion
- Anorexia
- Abdominal pain
How would you diagnose Wilm’s Tumour?
- US
- MRI
What is the most common bone tumour in young children?
Ewing’s Sarcoma
What is retinoblastoma?
Malignant tumour of the retinal cells which can arise from mutations in the RB1 gene or the MYCN gene.
How does retinoblastoma present?
White pupillary reflex
What is tumour lysis syndrome?
Refers to the constellation of metabolic disturbances that may follow the initiation of cancer treatment
How does tumour lysis syndrome occur?
Malignant cells have high turnover rate -> produce a greater amount of nucleic acid products (which transform to uric acid) and phosphate.
TLS is caused by the rapid destruction of tumour cells, usually in response to chemotherapy. Release of intracellular contents into bloodstream results in an elevation in serum levels:
- Uric acid
- Potassium
- Phosphate -> reduction in the level of calcium
In TLS, why does acute renal failure occur?
Kidney’s filtering ability becomes saturated
- Hyperuricaemia + acidic urine + reduced urinary flow = uric acid crystals + renal tubular obstruction + decline in renal function.
- Hyperphosphataemia = nephrocalcinosis + urinary obstruction
What are symptoms of tumour lysis syndrome in children?
After initial treatment - typically 7 days
- Nausea/Vomiting
- Anorexia/Lethargy
- Diarrhoea
- Muscle weakness/paraesthesia/cramps
- Laryngeal spasm.
What are signs of Tumour Lysis Syndrome?
- Related to characteristics/complications
- Hyper/hypotension
- Oliguria/anuria/haematuria
- Cardiac arrhythmias - syncope, chest pain, or dyspnoea
- Hypocalcaemia signs - tetany, Chvostek sign, Trousseau sign, or seizures
How would you manage a child who is showing signs of tumour lysis syndrome?
- ECG Monitoring
- Hyperhydrate - 2.5l/m2
- Diuresis
- Treat hyperkalaemia
- Watch urate and PO4
What is important to remember when treating a child with Tumour lysis syndrome?
NEVER GIVE THEM K+
How do you treat hyperkalaemia in the context of tumour lysis syndrome?
- Calcium gluconate - cardioprotective agent - reducing heart muscle excitability and resolving many changes in heart rhythm seen on an ECG
- Salbutamol - lowers blood levels of K+ by promoting its movement into cells
- Insulin/dextrose - drives potassium back into the cell
How would you manage increased urate levels in a child with Tumour lysis syndrome?
Decrease uric acid
- Allopurinol - acts on xanthine oxidase
- Rasburicase - acts as urate oxidase -> converts Uric acid to allantoin - more likely to use in context of large tumour
What is the definition of febrile neutropenia?
_Neutrophils < 0.5 x 109/L and Fever* > 38.0°C_
In addition, patients for whom there is a clinical suspicion of sepsis in the absence of fever should be treated as potentially bacteraemic e.g. unexplained abdominal pain or generally unwell.
How would you investigate suspected febrile neutropenia?
- Bloods - FBC, CRP, Serology
- Cultures - BCs, Swab, Stool, Urine
How would you treat febrile neutropenia?
- Piperacillin/tazobactam
- Meropenem - if penicillin allergy
- Add Gentamicin - if advised by consultant.
- Add Teicoplanin - if fever and/or rigors after line flushed earlier in day or soon after new line inserted
How would you manage a child with persistent febrile neutropenia after initial treatment?
Consider viral or fungal cause
- Aciclovir?
- Amphotericin?
What are features of cord compression in a child with a tumour?
- Ambulatory problems - Paraplegia
- Spine tenderness
- Spinchter distrubance
How would you treat cord compression in children with cancer?
- Dexamethasone
- Chemotherapy
- DXT - adjuvant radiotherapy
- Surgery
What is the abnormal red reflex seen in retinoblastoma called?
Leucocoria
What gene is at fault in retinoblastoma, and on what chromosome is it on?
RB1 gene on chromosome 13
How does retinoblastoma present?
- Leucocoria
- Squint
- Cellulitis
- Red Eye
- Painful eye
- Poor vision
What secondary malignancies can Retinoblastoma develop?
- Malignant melanoma
- Sarcoma
- Brain tumours
- Leukaemia
- Osteosarcoma
