18 - Oncology Flashcards
What are some differentials for bruising in children?
- Non-accidental injury
- Vascular disorders (e.g senile or simple purpura)
- Platelet disorders (e.g ITP, Leukaemia, Liver Disease).
- Coagulation disorders (Haemophilia, vit K deficiency, or von Willebrand disease).
- Drugs (e.g corticosteroids, warfarin, and alcohol)
What questions should you ask in the history about bruising?
- Any nose bleeds or menorraghia, could be platelet disorder
- Ask about possible underlying medical causes.
- Ask about alcohol and drugs
- Family history of a bleeding disorder, or a tendency to bruise or bleed easily
- Assessing the location and pattern of bruising in the context of the person’s age, mobility and developmental status, and the explanation for injury
What investigations need to be done for children with unexplained bruising (not suspected to be NAI)?
- Urgent FBC including platelet count (within 48 hours) to assess for leukaemia
- Blood film
- Clotting screen
What are some differentials for lymphadenopathy in children?
- Simple reactive to infection e.g tonsillitis
- Leukaemia
- Lymphoma
- Cervical lymphadenitis
- Glandular Fever
- Kawasaki Disease
- Drugs
- SLE
What are some drugs that can cause lymphadenopathy?
- Allopurinol
- Carbamazepine
- Lamotrigine
- Penicillin
- Phenytoin
- Sulfonamides
What questions should you ask in the history when a child has unexplained lymphadenopathy?
ALWAYS ASK ABOUT B SYMPTOMS
- Patient’s age
- Duration of mass
- Associated symptoms e.g fever, night sweats, weight loss
- Recent illness
- Drug history
- Exposure to animals, insects, anyone with TB
- Recent travel history
- Immunisation status
What examination should you do when a child has unexplained lymphadenopathy?
- Check child’s growth and development
- Size and quality of lesion
- Check all cervical lymph nodes, supraclavicular. axillary, inguinal to see if localised or generalised
- Look at eyes, ears, nose, mouth, throat as common causes of reactive lymphadenopathy
- Assess for rashes
- Assess for hepatosplenomegaly or other abdominal masses
What are some red flags for lymphadenopathy in children?
• >2cm
- Severe pallor
- Weight loss
- Loss of appetite
- Loss of energy
- Hard fixed non-tender mass
- Unexplained bruising or bleeding
• Unexplained fever or night sweats, unexplained weight loss, chest pain)
What are the most common malignancies associated with lymphadenopathy in children?
Worrying features: firm, indurated, fixed and matted lymph nodes that are usually not tender
<6: Acute leukaemia, Neuroblastoma, Rhabdomyosarcoma, Non-Hodgkin’s Lymphoma
>6: Non-Hodgkin’s Lymphoma and Rhabdomyosarcoma
If a child has reactive lymphadenopathy, what information should you give to the parent?
Reassure that this is a normal response to infection
Likely to slowly decrease over time, though this may take 2-4 weeks
It is normal for the lymph nodes to get bigger and smaller in future with intercurrent infections.
How does cervical lymphadenitis present and how is it treated?
- Enlarged tender lymph node with erythema
- Needs oral co-amoxiclav
- If >5cm or not improving send to hospital for IV antibiotics
When does lymphadenopathy need to be urgently referred in children?
Referral to rapid access clinic
•B symptoms
- Cervical lymphadenopathy >2cm and increasing in size or present for more than 2 weeks
- Unexplained inguinal lymphadenopathy >1.5cm
- Unexplained axillary, epitrochlear or supraclavicular lymph nodes > 1cm diameter
What investigations are done for suspected leukaemia/lymphoma because of the sign lymphadenopathy?
Bloods
- FBC
- Peripheral blood film
- C-reactive protein/ESR
- LFTs: for multisystem involvement
Imaging
- CT scan
- Biopsy of lymph node: via excision if FBC suggests malignancy
If blood malignancy is ruled out with lymphadenopathy what are some other blood tests you can do for a cause?
- EBV serology
- Bordella
- ASOT
What are some differentials for pallor in children?
- Anaemia
- Haemotological malignancy
- Shock e.g sepsis
What are causes of pancytopenia in children? (image important)
- Acute leukaemia
- Aplastic anaemia
- Infections e.g parvovirus B19, malaria
- Megaloblastic anaemia
- ITP
What are some clinical features of pancytopenia?
Think low RBC, WBC, platelets and the effects of that are pallor, infection and bleeding
What are the commonest childhood cancers?
- Leukaemia (ALL most common)
- CNS
- Lymphoma
What are the two main types of leukaemia in children and what age group are they most common in?
- Acute Lymphoblastic Leukaemia (ALL): 70-80% (B or T cell)
- Acute Myeloid Leukaemia (AML): 20%
What is the epidemiology and risk factors for ALL?
Epidemiology
- 800 cases a year
- Peak aged 2-5
- M>F
Risk Factors
- Radiation exposure e.g AXR during pregnancy
- Down’s syndrome
- Kleinfelter syndrome
- Noonan syndrome
- Fanconi’s anaemia
What is the pathophysiology of ALL?
Combination of genetic susceptibility and environmental exposure. Infection with virus may be trigger event
Proliferation of malignant lymphoid progenitor cells in the bone marrow in B-Cell (majority) or T-Cell Lineage
Proliferating malignant cells infiltrate and replace normal lines of haematopoietic cells in bone marrow resulting in their suppression so pancytopenia.
Infiltration can also occur in lymph nodes, liver, spleen
What are some cytogenetic associations with ALL?
- t(12;21): most common translocation, TEL-AML fusion gene
- t(9;22): Philadelphia chromosome. Associated with poor prognosis, BCR-ABL fusion gene.
- t(4;11): common in infants < 12 months
How may a child with ALL present?
Bone marrow failure
- Anaemia: pallor, tiredness, dizziness, SOB
- Infection: recurrent fever
- Thrombocytopenia: bruising, petechiae, bleeding e.g nose bleeds
General symptoms
- Lymphadenopathy
- Hepatosplenomegaly
- Muscular & bony pain
- Failure to thrive/Weight loss
- Night sweats
What are some some differentials for the symptoms in ALL?
- Bruising – ITP (low platelets but normal RBC/WBC), Trauma, NAI
- Recurrent infections – may be due to immune deficiency
- Lymphadenopathy – reactive lymphadenopathy
- Pancytopaenia – infiltration of other malignant processes into the marrow (eg neuroblastoma – abdominal mass may be present) or aplastic anaemia(lack of blasts on blood film).
What is the initial test if you suspect ALL in a child and what would the results show?
Urgent FBC within 48 hours
- Will have low RBC and Plt
- Will actually have raised WCC due to lymphocytosis but leukopenia
Blood Film
-May show blast cells in blood
What symptoms should prompt you to refer for urgent 48h FBC in children, and what symptoms need immediate referral?
- Unexplained bleeding or bruising
- Unexplained petechiae
- Persistent or unexplained bone pain
- Persistent fatigue
- Unexplained fever
- Unexplained generalised lymphadenopathy
- Hepatosplenomegaly (IMMEDIATE)
What further investigations should be done for leukaemia if FBC is abnormal?
- Bone marrow aspiration and biopsy: definitive, send cells for genetic analysis and immunophenotyping
- Lymph node biopsy
- Bloods:
- U+Es
- LFTs
- Clotting screen
- DDIMER
- Bone profile & Mg
- Uric acid: non specific tumour marker
- LDH: non specific tumour marker
- Blood borne virus screen
- Imaging: CXR (mediastinal mass), CT CAP, MRI head
Apart from a CT head, what other investigation can look fro neurological involvement in ALL?
Lumbar Puncture
What is the prognosis with ALL and what are some poor prognostic factors?
80% cure rate, better than AML
Risk Scoring
- Age – children aged 1-10 years at presentation have a better prognosis
- White cell count – WCC > 50 associated with poorer prognosis
- Gender - male
- CNS involvement
- Cytogenetics: philadelphia chromosome
How is ALL managed?
Immediate
- Resuscitating and stabilising unwell child
- Hyperhydration if high WCC to prevent hyper viscosity
- Steroids if mediastinal mass as risk of airway compromise
- Antibiotics if sepsis
Definitive and Long-term (UKALL 2011 Protocol)
- Chemotherapy IV, PO or Intrathecal
- Anti-fungals
- Blood products
- Tumour lysis prevention with steroids, allopurinol and IV hydration
- Maintenance treatment is 2 years for girls and 3 years for boys
- Possible stem cell transplant
What are the three treatment phases of ALL?
- Induction therapy
- Consolidation therapy
- Maintenance phase: for 2 years
What antimicrobial agent needs to be used during chemotherapy for ALL?
- Co-trimoxazole prevents pneumocystosis
What are some complications with ALL?
- Tumour lysis syndrome: give prophylaxis
- Neutropenic sepsis: ever and neutrophils < 0.5, medical emergency
- SVCO: due to mediastinal mass
- Chemotherapy side-effects: early (e.g. mucositis, N+V) hair loss) or late (e.g. cardiomyopathy, secondary malignancies, infertility, stunted growth)
Why is allopurinol given to prevent tumour lysis syndrome?
Prevents hyperuricaemia
How do you manage a child with suspected neutropenic sepsis?
- Take blood cultures and MSU, swab all orifices
- Do FBC, CRP & serology
- Give Tazocin
- Consider aciclovir if not improving and negative blood cultures
What is the epidemiology of lymphoma in children?
- More common in older children
- More common in boys
- 10% of childhood cancers
- More commonly Non-Hodgkin’s Lymphoma
What are some risk factors for lymphoma in children?
Genetic susceptibility, Infection and Environment Exposure
- EBV
- Immunosuppresssed patients
- Previous history of a cancer
What are the clinical features of lymphoma?
- Generalised lymphadenopathy: non-tender rubbery
- B Symptoms: fever, weight loss, night sweats
- Splenomegaly
- Fatigue
- Pruritis
- Shortness of Breath
Lymphomas can affect mediastinal lymph nodes, how may this present?
- Wheeze
- Cough
- SOB
- SVCO
What are some differentials for lymphadenopathy in lymphoma?
- Reactive lymphadenopathy
- Leukaemia: think if anaemia and pallor
- Metastatic malignancy
What referral takes place if there are any red flags in children for lymphoma?
Urgent referral for specialist appointment within 48 hours (2 weeks in adults)
Bloods
- FBC
- U+Es
- LDH
Imaging
- USS and Lymph Node Biopsy
- CXR: symptoms of mediastinal node involvement
- Full body CT for staging
How is lymphoma staged?
Ann Arbour
Presence of B symptoms is worse prognosis
- Stage 1: single group of lymph nodes or a single organ
- Stage 2: 2 or more groups of lymph nodes or organs on same side of diaphragm
- Stage 3: present in lymph nodes or organs on both sides of the diaphragm
- Stage 4: Diffuse involvement of lymph nodes and organs such as the liver and bones
How is lymphoma in children managed?
Immediate
- High dose steroids and airways support mediastinal mass
- Tumour lysis syndrome: hyperhydration and allopurinol
Definitive and Long‐term
- Chemotherapy: risk of leukaemia and infertility
- Possibly radiotherapy: risk of cancer to tissues/skin
What is the prognosis with lymphoma?
The majority of children and young people with lymphoma will recover completely, with Hodgkin’s lymphoma carrying a more favourable prognosis
Brain tumours are the most common solid cancer in children. What are the most common types of brain tumours?
- Astrocytoma [40%]
- Medulloblastoma[13%]
- Ependymoma [7%]
- Craniopharyngioma[5%]
- Germ cell tumours [4%]
How may a brain tumour in children present?
History
- Headache: Due to mass effect or hydrocephalus from blockage of CSF flow
- Nausea / Vomiting: due to raised ICP
- Behavioural change: frontal lobe tumours
- Polyuria/polydipsia
- Seizures
- Altered GCS
Examination
- Visual symptoms: diplopia, reduced visual acuity/visual fields, abnormal eye movement/fundoscopy
- Motor signs: abnormal gait or coordination, swallowing difficulties, weakness
- Delayed growth
- Delayed, arrest or precocious puberty
- Increased head circumference if under 2 years old
How are brain tumours in children diagnosed and managed?
Dx
- MRI head
Mx
- Initial management analgesia, antiemetics, anticonvulsants, fluid/dietary support, and treatment to lower ICP such as steroids.
- Surgical resection
- CSFs shunts: if hydrocephalus
- Radiotherapy: can be adjuvant to surgical resection
- Chemotherapy: commonly used in situations where the tumour cannot be removed completely with surgery.
- Proton therapy
What is a neuroblastoma and how may it present in children?
Malignancy of neural crest cells of sympathetic NS
- Most common cancer in under 1’s
- Presents late: 60% have metastases at diagnosis
- Wide range of non-specific symptoms
- Mid line abdominal mass and weight loss are most common symptoms
What is Wilm’s tumour and what is it associated with?
- Nephroblastoma
- Commonest intra-abdominal tumour
- Overgrowth: hemihypertrophy, Beckwith-Wiederman
How is Wilm’s tumour diagnosed and managed?
- Flank pain, abdominal mass, haematuria
- Diagnosis: USS, biopsy and staging
- Nephrectomy & chemotherapy +/- radiotherapy
- Survival 80-90%
How does retinoblastoma present and how is it treated?
Leukocoria and Squint
What are the most common bone tumours in children and how do they present?
- Osteosarcoma (most common)
- Ewing’s Sarcoma
NICE guidelines advise that children, teenagers and young adults with an unexplained bony lump should be referred for an urgent x-ray within 48 hours. If an x-ray suggests possible bone cancer then an urgent referral for an appointment within 48 hours for specialist assessment should be made
