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PAEDS ONCOLOGY/HAEMATOLOGY Flashcards

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1
Q

BRAIN TUMOURS
What is the site of brain tumours?

A
  • Almost always primary (unlike adults)
  • 60% are infratentorial
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2
Q

BRAIN TUMOURS
What are the different types of brain tumours?

A
  • Astrocytoma (#1) varies from benign to glioblastoma multiforme
  • Medulloblastoma arises in the midline of posterior fossa, may have spinal mets
  • Ependymoma mostly in posterior fossa where it behaves as medulloblastoma
  • Brainstem glioma
  • Craniopharyngioma
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3
Q

BRAIN TUMOURS
What is a craniopharyngioma?
How does it present?

A
  • Developmental tumour arising from squamous remnant of Rathke pouch
  • Not truly malignant but locally invasive (bitemporal hemianopia often lower quadrant as superior chiasmal compression)
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4
Q

BRAIN TUMOURS
What is the clinical presentation of brain tumours?

A
  • Evidence of raised ICP
  • Focal neurology dependant on where the lesion is
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5
Q

BRAIN TUMOURS
What are some signs of raised ICP?

A
  • Headache worse in morning
  • Papilloedema
  • Vomiting, esp. in the morning
  • Behaviour or personality change
  • Visual disturbance (squint secondary to 6th nerve palsy, nystagmus)
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6
Q

BRAIN TUMOURS
What are some focal neurological signs?

A
  • Spinal tumours = back pain, peripheral weakness of arms/legs or bladder + bowel dysfunction depending on level of lesion
  • Ataxia, seizures
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7
Q

BRAIN TUMOURS
What is the best investigation for brain tumours?

A
  • MRI for visualisation
  • Avoid LP if raised ICP
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8
Q

BRAIN TUMOURS
What are some complications of brain tumours?

A
  • Outcome depends on location, how much is cleared + how much healthy brain tissue removed
  • Survivors face neuro disability, growth + endocrine problems, neuropsychological issues
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9
Q

BRAIN TUMOURS
What is the management of brain tumours?

A
  • 1st line = surgical resection + ventriculoperitoneal shunt to reduce risk of coning + treat hydrocephalus
  • Chemo (fewer options as less drugs cross BBB) or radiotherapy
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10
Q

NEUROBLASTOMA
What is a neuroblastoma? Epidemiology?

A
  • Arise from neural crest tissue in the adrenal medulla + sympathetic nervous system,
  • most common <5y,
  • NOT brain tumour
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11
Q

NEUROBLASTOMA
Why are neuroblastomas biologically unusual?
What types of neuroblastomas are there?

A
  • Spontaneous regression sometimes occur in v young infants
  • Spectrum from benign (ganglioneuroma) to highly malignant neuroblastoma
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12
Q

NEUROBLASTOMA
What is the clinical presentation of neuroblastoma?

A
  • Abdominal mass
  • Sx of metastatic = weight loss, hepatomegaly, pallor, bone pain + limp
  • Uncommon = paraplegia, cervical lymphadenopathy, proptosis, periorbital bruising, skin nodules
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13
Q

NEUROBLASTOMA
How does the abdominal mass present?

A
  • Often crosses midline + envelopes major vessels + lymph nodes
  • Can grow very large
  • Classically abdo primary is of adrenal origin
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14
Q

NEUROBLASTOMA
What are the investigations for neuroblastoma?

A
  • Raised urinary catecholamine levels
  • CT/MRI + confirmatory biopsy
  • Evidence of metastatic disease = bone marrow sampling, MIBG scan ±bone scan
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15
Q

NEUROBLASTOMA
What is the management of neuroblastoma?

A
  • Localised primaries + no mets can often be cured with surgery
  • Metastatic = chemo, autologous stem cell rescue, surgery + radio
  • Immunotherapy may be used for long-term maintenance
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16
Q

WILM’S TUMOUR
What is a Wilm’s tumour?
Epidemiology?
Risk factor?

A
  • Nephroblastoma that originates from embryonal renal tissue, cure rate 80%
  • > 80% present before age 5, rare after 10y
  • FHx = Wilm’s tumour susceptibility gene
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17
Q

WILM’S TUMOUR
What is the clinical presentation of a Wilm’s tumour?

A
  • Large abdominal mass found incidentally in an otherwise well child
  • May have flank pain, anorexia, anaemia, painless haematuria + HTN
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18
Q

WILM’S TUMOUR
What are the investigations for Wilm’s tumour?

A
  • USS ± CT/MRI showing intrinsic renal mass distorting the normal structure
  • Mass with characteristic mixed tissue densities (cystic + solid)
  • Staging for distant mets (often lung), biopsy for histology Dx
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19
Q

WILM’S TUMOUR
What is the management of a Wilm’s tumour?

A
  • Initial chemo followed by delayed nephrectomy (full if 1 kidney, partial if bilateral but RARE)
  • Radiotherapy for more advanced disease
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20
Q

BONE TUMOURS
What are bone tumours?
When do they occur?

A
  • Osteogenic sarcoma more common than Ewing sarcoma but Ewing seen more in younger children
  • Both have male predominance
  • Uncommon before puberty
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21
Q

BONE TUMOURS
What is the clinical presentation of bone tumours?

A
  • Limbs most common site (particularly femur, tibia + humerus)
  • Persistent localised bone pain often precedes mass, otherwise well
  • May be worse at night + cause disrupted sleep
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22
Q

BONE TUMOURS
What are some investigations for bone tumours?

A
  • Raised ALP on bloods
  • Plain XR followed by MRI + bone scan, ?PET scan + bone biopsy
  • CT chest for lung mets + bone marrow sampling to exclude involvement
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23
Q

BONE TUMOURS
How might bone tumours present on radiographs?

A
  • XR = destruction + variable periosteal new bone formation
  • Periosteal reaction leads to classic “sunburst” appearance
  • Ewing sarcoma often shows substantial soft tissue mass
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24
Q

BONE TUMOURS
What is the management for bone tumours?

A
  • Combo chemo before surgery (amputation avoided if possible)
  • Radio used in Ewing sarcoma for local disease, esp. if surgical resection is impossible or incomplete (e.g. pelvis or axial skeleton)
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25
RETINOBLASTOMA What is a retinoblastoma?
- Malignant tumour of retinal cells which can lead to severe visual impairment
26
RETINOBLASTOMA What is a genetic cause of retinoblastoma? How might it present?
- Retinoblastoma susceptibility gene on chromosome 13 = AD but incomplete penetrance > offer genetic screening - All bilateral tumours are hereditary, 20% of unilateral are
27
RETINOBLASTOMA What is the clinical presentation of retinoblastoma?
- White pupillary reflex noted to replace the normal red reflex or a squint - May have decreased visual acuity + nystagmus - Most present within 3y
28
RETINOBLASTOMA What are the investigations for retinoblastoma?
- Screened for in NIPE - MRI + Examination under anaesthetic - Biopsy is avoided + treatment based on ophthalmological findings
29
RETINOBLASTOMA What are some complications of retinoblastoma?
- Significant risk of second malignancy (especially sarcoma) amongst survivors of hereditary retinoblastoma
30
RETINOBLASTOMA What is the management aims for retinoblastoma? What is the management?
- Cure cancer + preserve vision - Chemo, particularly if bilateral to shrink tumours > local laser treatment to retina - Radiotherapy or enucleation of eye (removal) if advanced - Most cured but many visually impaired
31
LIVER TUMOURS What are liver tumours? In neonates?
- Mostly hepatoblastoma or hepatocellular carcinoma - Primary liver tumours in neonates = haemangioma
32
LIVER TUMOURS What is the clinical presentation of liver tumours?
- Abdominal distension or mass are common - Pain + jaundice rare
33
LIVER TUMOURS What are the investigations for liver tumours?
- Elevated serum alpha fetoprotein in nearly all cases - USS/CT/MRI to visualise the tumour + extent of disease
34
LIVER TUMOURS What is the management of liver tumours?
- Chemo, surgery or liver transplant if inoperable - Majority of hepatoblastoma can be cured but prognosis worse for hepatocellular
35
FANCONI SYNDROME What is fanconi syndrome?
- Generalised reabsorptive disorder of renal tubular transport in the PCT resulting in... – Type 2 (proximal) renal tubular acidosis – Polydipsia, polyuria, aminoaciduria + glycosuria – Osteomalacia/rickets
36
FANCONI SYNDROME What are some causes of fanconi syndrome?
- Usually secondary to inborn errors of metabolism – Cystinosis (AR > intracellular accumulation of cysteine, most common) – Wilson's disease, galactosaemia, glycogen storage disorders
37
FOETAL HAEMOGLOBIN What is haemoglobin? What is the structural difference between foetal and adult haemoglobin?
- Responsible for transporting oxygen around the body in RBCs - HbF = 2 alpha + 2 gamma subunits - HbA = 2 alpha + 2 beta subunits
38
FOETAL HAEMOGLOBIN What is the main difference between HbF + HbA?
- HbF has greater affinity to oxygen than adult so oxygen binds more easily + is more reluctant to let go = crucial for oxygen to transport from maternal to foetal Hb
39
FOETAL HAEMOGLOBIN When is Hb concentration highest? When does the shift from HbF to HbA occur?
- At birth to compensate for low oxygen concentration in the foetus - By 6m of age very little HbF produced so HbA predominates
40
ANAEMIA OVERVIEW What is anaemia? How is it defined in paeds?
- Hb level below the normal range - Neonate = <14g/dL - 1–12m = <10g/dL - 1–12y = <11g/dL
41
ANAEMIA OVERVIEW What are the 3 main mechanisms of anaemia?
- Increased red cell production - Increased red cell destruction (haemolysis) - Blood loss (uncommon in paeds like Meckel's, vWD, foetomaternal bleeding)
42
ANAEMIA OVERVIEW What are some causes of decreased red cell production? What are some clues?
- Ineffective erythropoiesis (Fe, folate deficiency, CKD) - Red cell aplasia - Normal reticulocytes, abnormal MCV in nutrient deficiencies
43
ANAEMIA OVERVIEW What are some causes of haemolysis? What are some clues?
- G6PD deficiency, haemoglobinopathies, hereditary spherocytosis - Raised reticulocytes, abnormal appearance on blood films, +ve direct antiglobulin test if immune cause
44
ANAEMIA OVERVIEW What is haemolytic anaemia? What is the normal lifespan of RBC?
- Characterised by reduced red cell lifespan due to increased red cell destruction in the circulation (intravascular haemolysis) or liver/spleen (extravascular) - 120d
45
ANAEMIA OVERVIEW How does haemolysis cause anaemia? What is the difference in haemolytic anaemias in neonates + children?
- Red cell survival reduced significantly but bone marrow production increases too, anaemia = bone marrow cannot compensate - Neonates = immune haemolytic anaemias, children = instrinsic abnormalities (G6PD)
46
ANAEMIA OVERVIEW List 4 features of haemolytic anaemias
- Anaemia - Hepatosplenomegaly - Unconjugated bilirubinaemia - Excess urinary urobilinogen
47
ANAEMIA OVERVIEW What are some causes of anaemia in the neonate?
- Reduced RBC production = congenital red cell aplasia + congenital parvovirus infection > red cell aplasia - Haemolytic anaemia = immune (haemolytic disease of newborn) or hereditary (G6PD etc)
48
ANAEMIA OVERVIEW What are the main causes of anaemia of prematurity?
- Inadequate erythropoietin production - Reduced red cell lifespan - Frequent blood sampling whilst in hospital - Iron + folic acid deficiency after 2-3m.
49
IRON DEF ANAEMIA What is iron deficiency anaemia? Why does it cause anaemia? Iron physiology?
- #1 cause of childhood anaemia - Bone marrow requires iron to produce Hb - Iron mainly absorbed in the duodenum + jejunum + requires acid from the stomach to keep iron in soluble ferrous (Fe2+) form, if acid drops it changes to insoluble ferric (Fe3+) form
50
IRON DEF ANAEMIA What are some causes of iron deficiency anaemia?
- Inadequate intake = common as infants require additional iron for increasing blood volume - Malabsorption = Crohn's + coeliac - Blood loss = common in menstruating females
51
IRON DEF ANAEMIA What are some sources of iron? What can affect iron absorption?
- Breast milk, formula, cow's milk or weaning (cereals) - Markedly increased when eaten with food rich in vitamin C + inhibited by tannin in tea
52
IRON DEF ANAEMIA What are the symptoms of iron deficiency anaemia?
- Generic = fatigue, SOB, headaches, dizziness, palpitations - Young infants feed more slowly + children tire easily
53
IRON DEF ANAEMIA What are some signs of iron deficiency anaemia?
- Generic = pallor (inc. conjunctival), tachycardia, tachypnoea - Pica = consumption of non-food materials - Koilonychia, angular cheilitis, brittle hair + nails
54
IRON DEF ANAEMIA What are some investigations for iron deficiency anaemia and what would you see?
- FBC = low Hb, microcytic (low MCV + MCH), normal reticulocytes - Blood film = hypochromic microcytic red cells - Iron studies: – Low = serum ferritin, iron + transferrin saturation – High = total iron binding capacity
55
IRON DEF ANAEMIA What is... i) transferrin saturation? ii) total iron binding capacity?
i) Proportion of transferrin bound to iron ii) Total space on transferrin for Fe to bind
56
IRON DEF ANAEMIA What is the management of iron deficiency anaemia?
- Diet = eat red meat (beef, lamb), oily fish, green veg (broccoli, spinach), dried fruit (raisins) - Children may be given polysaccharide iron complex (Niferex) - Ferrous sulfate or fumarate often used
57
IRON DEF ANAEMIA What are some side effects of treatment with oral iron supplementation?
- Constipation - Black coloured stools - Nausea
58
VON WILLEBRAND DISEASE What is the physiological role of von Willebrand factor?
- Facilitates platelet adhesion to damaged endothelium - Acts as carrier protein for FVIII:C, protecting it from inactivation + clearance
59
VON WILLEBRAND DISEASE What is von Willebrand disease (vWD)? What causes it? Types?
- Deficiency of vWF leading to defective platelet plug formation + deficient FVIII:C > most common inherited bleeding disorder - AD, type 1 most common + mildest - Severity increases with type 2, type 3 has very low or no vWF (AR)
60
VON WILLEBRAND DISEASE What is the clinical presentation of vWD?
- Bruising, excessive + prolonged bleeding after surgery, mucosal bleeding (epistaxis, menorrhagia, bleeding gums) - In contrast to haemophilia = spontaneous soft tissue bleeding like large haematomas uncommon
61
VON WILLEBRAND DISEASE What are some investigations for vWD?
- FBC (normal platelets) + blood film, biochemical screen including renal + liver function - Prolonged bleeding time - Prothrombin time normal - APTT = elevated or normal - vWF antigen decreased, vWF multimers variable
62
VON WILLEBRAND DISEASE What is the management of vWD?
- Pressure applied if active bleeding - Minimise bleeding with desmopressin or TXA - Severe = plasma derived FVIII concentrate or vWF infusion - AVOID aspirin, NSAIDs + IM injections as can worsen bleeding
63
VON WILLEBRAND DISEASE How is desmopressin given? What does it do?
- Nasal or s/c - Release of vWF + FVIII concentrate
64
COAGULATION DISORDERS What are acquired disorders of coagulation?
Secondary to - Haemorrhagic disease of the newborn due to vitamin K deficiency - Liver disease as location of clotting factor production - ITP + DIC
65
COAGULATION DISORDERS What is vitamin K essential for? What does deficiency result in? How can this be prevented?
- Essential for factors 2, 7, 9 + 10 (1972) production + naturally occurring anticoagulants like protein C + S - Prolonged prothrombin time so increased bleeding - All neonates get vitamin K IM to facilitate coagulation
66
COAGULATION DISORDERS What can cause vitamin K deficiency?
- Inadequate intake = neonates, long-term chronic illness - Malabsorption = coeliac, cystic fibrosis - Vitamin K antagonists = warfarin
67
ITP What is immune thrombocytopenic purpura (ITP)?
- Commonest cause of thrombocytopenia in childhood - T2 hypersensitivity reaction with destruction of circulating platelets by anti-platelet IgGs
68
HAEMOLYTIC DISEASE OF THE NEWBORN what is the pathophysiology?
- rhesus positive father and negative mother have a rhesus positive baby - mother + baby have incompatible antigens which induces primary immune response - problems tend to occur in subsequent pregnancies + results in destruction of foetal haemoglobin
69
HAEMOLYTIC DISEASE OF THE NEWBORN what is the clinical presentation?
- anti-D antibodies in mother detected by Coombe's test that all women have at 1st antenatal appointment - routine USS may detect hydrops fetalis or polyhydramnios - mild cases = jaundice, pallor + hepatosplenomegaly, hypoglycaemia - severe cases = oedema, petechiae + ascites
70
HAEMOLYTIC DISEASE OF THE NEWBORN what are the investigations?
- indirect coombe's test show antibodies - antenatal USS shows hydrops fetalis - fetal blood sample
71
HAEMOLYTIC DISEASE OF THE NEWBORN what is the management in utero?
- transfusion of O negative packed cells cross-matched with maternal blood at 16-18 weeks
72
HAEMOLYTIC DISEASE OF THE NEWBORN what is the management after delivery?
50% = normal haemoglobin + bilirubin but should be monitored for anaemia for 6-8 weeks 25% = require transfusion + may require phototherapy to avoid kernicterus 25% = stillborn or have hydrops fetalis
73
HAEMOLYTIC DISEASE OF THE NEWBORN what are the complications?
- kernicterus which can cause extrapyramidal, auditory and visual abnormalities and cognitive deficit - late-onset anaemia - graft-versus-host disease - portal vein thrombosis + portal hypertension
74
HAEMOLYTIC DISEASE OF THE NEWBORN how can it be prevented?
identify all women who have been sensitised by coombe's testing at first antenatal visit anti-D immunoglobulin should be given to all rhesus negative women at 28 + 34 weeks
75
NEUROBLASTOMA Where is it located?
Mass anywhere along sympathetic chain so could lead to spinal cord compression
76
FOETAL HAEMOGLOBIN When does HbF production reduce?
HbF production decreases from 32w with HbA + HbA2 production increasing

MY REVISION - ALL PHASES (36 decks)

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