Oncology, Neurology Flashcards

1
Q

How is childhood cancer classified?

A

ICCC (International classification of childhood cancer)

Based on tumour morphology (cell origin) and primary site

Standard classification is essential for comparing incidence & survival across regions & time periods

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

When are children more likely to get cancer?

A

At a younger age (0-4yo)

15-24 yo = another peak as they have an increased risk of germ cell tumours and lymphomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

In 10% what are possible causes that may be identified for cancer?

A
  • Genes (Down, Fanconi, BWS, Li-Fraumeni familial cancer syndrome, neurofibromatosis)
  • Environment (radiation, infection(e.g. EBV))
  • Iatrogenic (RT, chemo)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Is child cancer sporadic?

A

Usually yes but it is important to ask about risk factors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What makes the categories for referral in child cancer?

A

IMMEDIATE=unexplained petechiae, hepatosplenomegaly

URGENT=repeated attendance, same problem, no clear diagnosis OR new neuro symptoms, abdo mass

REFER (to doctor or for urgent investigation)=rest pain, back pain & unexplained lump, lymphadenopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are some examples of oncological emergencies?

A
  • Sepsis/febrile neutropenia
  • Raised ICP
  • Spinal cord compression
  • Mediastinal mass
  • Tumour lysis syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are risks for sepsis/febrile neutropenia?

A

ANC< 0.5 x10 to the 9
Indwelling catheter
Mucosal inflam
High dose chemo/SCT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How do those with sepsis/febrile neutropenia present?

A
  • Fever (or low temp)
  • Rigors
  • Drowsiness
  • Shock (metabolic acidosis)

If child is not responding to simple measures and has significant shock sometimes they need ionotropic support to help their BP, transferred to PICU

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the early presentation of raised ICP?

A
  • Early morning headache/vomiting
  • Tense fontanelle
  • Increasing HC (head circumference)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the signs of late presentation of raised ICP?

A
  • Constant headache
  • Papilloedema
  • Diplopia (VI palsy)
  • Loss of upgaze
  • Neck stiffness
  • Status epilepticus
  • Reduced GCS
  • Cushing’s triad (low HR, high BP, falling RR)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the investigation for raised ICP?

A

Imaging mandatory if safe

CT is good for screening

MRI is best for more accurate diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the management of raised ICP?

A

DEXAMETHASONE if due to tumour (reduce oedema & increase CSF flow)

NEUROSURGERY-urgent CSF diversion
- Ventriculostomy-hole in membrane at base of 3rd ventricle with endoscope
- EVD (temporary)
- VP shunt

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

In whom is spinal cord compression more common (potential complication of nearly all paed malignancies)?

A

Ewing’s or Medulloblastoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

When does spinal cord compression arise and what is the pathological process of it?

A

Diagnosis of cancer(65%), relapse, progression

  • Invasion from paravertebral disease via intervertebral foramina (40% extradural)
  • Vertebral body compression (30%)
  • CSF seeding
  • Direct invasion
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the presentation of spinal cord compression?

A

Symptoms vary with level

  • WEAKNESS
  • PAIN
  • SENSORY
  • SPHINCTER DISTURBANCE
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How is spinal cord compression managed?

A
  • URGENT MRI
  • Start DEXAMETHASONE urgently to reduce peri-tumour oedema
  • Definitive treatment with chemo is appropriate when rapid response is expected (surgery or RT are other options)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What does the outcome of spinal cord compression depend on?

A
  • Severity of impairment rather than the duration between symptoms and diagnosis

(Mild impairment >90% recovery, Paraplegic 65% recovery)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

SVC syndrome (or SMS) is rare (<1% of new paediatric malignancies): what are the common causes of it?

A
  • LYMPHOMA
  • Other=neuroblastoma, germ cell tumour, thrombosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

How does SVC syndrome and SMS present and how is it investigated?

A

SVCS: facial, neck and upper thoracic plethora, oedema, cyanosis, distended veins, ill, anxious, reduced GCS

SMS: dyspnoea, tachypnoea, cough, wheeze, stridor, orthopnoea

Investigation:
CXR / CT chest (if able to tolerate)
Echo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the management of SVCS and SMS?

A

Keep upright & calm
Urgent biopsy (ideally)

Look to obtain important diagnostic information without GA
FBC, BM, pleural aspirate, GCT markers

Definitive treatment is required urgently:
- Chemotherapy is usually rapidly effective
- Presumptive treatment may be needed in the absence of a definitive histological diagnosis (steroids)

  • RT is effective (May cause initial increased respiratory distress)
  • Rarely surgery if insensitive
  • CVAD-associated thrombosis should be treated by thrombolytic therapy

Most of underlying malignancies have a good prognosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is tumour lysis syndrome caused by?

A

Metabolic derangement

Rapid death of Tumour Cells

Release of intracellular contents

At or shortly after presentation

Secondary to treatment

(rarely spontaneous)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are the clinical features of tumour lysis syndrome?

A

INCREASED potassium, urate-relatively insoluble, phosphate

REDUCED calcium

Acute renal failure:
Urate load
CaPO4 deposition in renal tubules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is the treatment of tumour lysis syndrome?

A

Avoidance
ECG Monitoring
Hyperhydrate-2.5l/m2
QDS electrolytes
Diuresis

NEVER GIVE POTTASIUM (or phosphate)

Decrease uric acid:
- Urate Oxidase-uricozyme (rasburicase)-acute setting
- Allopurinol

Treat hyperkalaemia:
Ca Resonium
Salbutamol
Insulin

Renal replacement therapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What do oncologists use to see what cancer is and what harm it is causing?

A
  • Scans
  • Biopsy/pathology
  • Cytogenetics
  • Tumour markers
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What tumours can secrete AFP?
Liver tumours or germ cell tumours Can be diagnostic
26
How is cancer staged?
Staging-e.g. scans, bone marrow CXR or CT chest Bone scan Number of different tests depending on what's the primary tumour and the location of the primary tumour
27
What is cannonball metastases in the chest typical of?
Sarcoma diagnosis-indicate very poor prognosis
28
What does treatment of cancer depend on?
Multimodal therapy based on specific disease and extent (plus patient factors) MDT approach National/international collaboration Clinical research
29
How does a bone marrow transplant work?
Collect STEM cells from somebody else and put them back into a new patient where they will travel to the bone marrow and then set up a completely new immune system. Bone marrow transplants allow you to use very high dose chemotherapy but they also give you an immune response where the donor immune system will recognise the host cancer cells as foreign and kill them using the same kind of approach as immunotherapy.
30
What are the acute risks of chemo?
Hair loss Nausea & vomiting Mucositis Diarrhoea / constipation Bone marrow suppression – anaemia, bleeding, infection
31
What are the chronic risks of chemo?
Organ impairment – kidneys, heart, nerves, ears Reduced fertility Second cancer
32
What are the acute and chronic risks of RT?
Acute: Lethargy Skin irritation Swelling Organ inflammation – bowel, lungs Chronic: Fibrosis / scarring Second cancer Reduced fertility
33
Survivors of childhood cancer have ... toxicity
ACCUMULATIVE (from being treated with childhood cancer)
34
How so is child neurology dynamic?
Brain continues to grow and brain functions evolve Neurodevelopment continues to progress Static lesion produce evolving features
35
Why is time course of symptoms crucial in a neurological condition?
To ascertain symptom progression - and to try to determine a differential diagnosis Distinguishing static from slowly progressive symptoms can be challenging
36
What is crucial to ask in a developmental Hx?
- Motor milestones (gross and fine motor skills) - Speech and language development - Play esp symbolic play and social behaviour - Self help skills - Vision and hearing assessment Global delay=if delayed in 2 or mote of these domains Disordered development=1 of these fields
37
What is included in the neurological examination in childhood?
- Opportunistic approach and observation skills - Appearance - Gait - Head size - Skin findings (neurocutaneous markers (e.g. Cafe au lait spots---neurofibromatosis, Port wine stain---Sturge Weber syndrome)
38
What neurological conditions are seen (examples)?
Migraine Traumatic brain injury Tourette syndrome (1% of all children with high frequency in ADHD & OCD) Epilepsy Brain tumours (2nd most common cancer in children)
39
What are the categories of headache a child can have?
- Isolated acute - Recurrent acute - Chronic progressive - Chronic non-progressive
40
How would you classify a chronic headache?
Patient suffers from a sore head for at least 15 days out of 30 days in a month
41
What should be included in a recurrent or chronic headache Hx?
- Is there more than 1 type of headache Typical episode - Any warning - Location - Severity - Duration - Frequency
42
What should be included in a headache examination?
- Growth parameters, OFC, BP (HT can be a signal of intracranial pathology but can also itself cause headaches) - Sinuses, teeth, visual acuity - Fundoscopy - Visual fields (craniopharyngioma) - Cranial bruit - Focal neurological signs - Cognitive and emotional status The diagnosis of headache etiology is clinical
43
What pointers would point you towards childhood migraine?
Hemicranial pain - throbbing/pulsatile Associated abdominal pain, nausea, vomiting Focal symptoms/ signs before, during, after attack: Visual disturbance, paraesthesia, weakness (AURA) ‘Pallor’ Aggravated by bright light/ noise (Photophobia/phonophobia) Relation to fatigue/ stress Helped by sleep/ rest/ dark, quiet room Family history often positive
44
What pointers would make you think of a tension headache (as apposed to migraine)?
- Diffuse, symmetrical - Band like distribution - Present most of the time (but there may be symptom free periods) - 'constant headache'
45
Pointers to raised ICP (Red flags)?
Aggravated by activities that raise ICP eg. Coughing, straining at stool, bending Woken from sleep with headache +/- vomiting
46
What presentation features would make you think of analgesic overuse headaches?
Headache is back before allowed to use another dose Paracetamol/ NSAIDs Particular problem with compound analgesics eg. Cocodamol
47
In headache what are the indications for neuroimaging?
Features of cerebellar dysfunction Features of raised ICP New focal neurological deficit eg. new squint Seizures, esp focal Personality change Unexplained deterioration of school work
48
How is migraine managed (Acute & Preventative)?
Acute attack: effective pain relief, triptans Preventative (at least 1/week): Pizotifen, Propranolol, Amitryptyline, Topiramate, Valproate
49
How is TTH managed?
Aim at reassurance: no sinister cause Attention to underlying chronic physical, psychological or emotional problems Acute attacks: simple analgesia Prevention: Amitryptiline Discourage analgesics in chronic TTH
50
What is the difference between a seizure/fit, syncope and convulsion and an epileptic seizure (all paroxysmal events)?
Seizure/ Fit: Any sudden attack from whatever cause Syncope: Faint (a neuro-cardiogenic mechanism) Convulsion: Seizure where there is prominent motor activity Epileptic seizure: an electrical phenomenon Seizures can have different mechanisms Many seizures are not epileptic in nature
51
What is an epileptic seizure and what does it depend on?
Epileptic seizure=An abnormal excessive hyper synchronous discharge from a group of (cortical) neurons It may have clinical manifestations Paroxysmal change in motor, sensory or cognitive function Depends on seizure’s location, degree of anatomical spread over cortex, duration
52
What is epilepsy?
Epilepsy: A tendency to recurrent, unprovoked (spontaneous) epileptic seizures A question that must be answered clinically, with recourse to EEG only for supportive evidence
53
Is a seizure always epilepsy?
NOOOOO A seizure is not necessarily epileptic Consequences of misdiagnosis of epilepsy can be serious
54
What are some examples of non epileptic seizures and other mimics in children?
Acute symptomatic seizures: due to acute insults eg. Hypoxia-ischaemia, hypoglycemia, infection, trauma Reflex anoxic seizure: common in toddlers (vagal overstimulation-provoked/triggered) Syncope Parasomnias eg. night terrors Behavioural stereotypies (repetitive movements) Psychogenic non-epileptic seizures (PNES)
55
What is a febrile convulsion?
An seizure occurring in infancy/ childhood, usually between 3 months and 5 years of age, associated with fever but without evidence of intracranial infection or defined cause for the seizure Commonest cause of ‘acute symptomatic seizure’ in childhood
56
What are the seizure types (can be challenging to distinguish between)?
Jerk/ shake: clonic, myoclonic, spasms Stiff: usually a tonic seizure Fall: Atonic/ tonic/ myoclonic Vacant attack: absence, focal absence seizure
57
What is the mechanism of an epileptic fit?
Chemically triggered by chemical imbalance: - Decreased inhibition (gama-amino-butyric acid, GABA) - Excessive excitation (glutamate and aspartate) - Excessive influx of Na and Ca ions Chemical stimulation produces an electrical current Summation of a multitude of electrical potentials results in depolarization of many neurons which can lead to seizures, can be recorded from surface electrodes (Electroencephalogram (EEG))
58
What are the main two types of epileptic seizures?
Generalised seizure (neurons recruited from both halves of the brain) Partial/focal seizure (restricted to one hemisphere or part of one hemisphere)
59
What is the difference between childhood vs adult onset epilepsies?
Majority are idiopathic in origin (both Focal & Generalised) Majority of epilepsies are generalised Seizures can be subtle (absences, myoclonus, drop attacks) Diagnosis can be challenging because: - Non-epileptic paroxysmal disorders are more common in children - Difficulty in explaining (Children are not young adults) - Difficulty in interpretation (witness) - Difficulty in interpretation and synthesising information(physician)
60
What is the stepwise approach to a diagnosis of epilepsy?
Is the paroxysmal event epileptic in nature? Is it epilepsy? What seizure types are occurring? What is the epilepsy syndrome? What is the etiology? What are the social and educational effects on the child?
61
How is an EEG valuable in epilepsy and how is it not?
An interictal EEG has limited value in deciding when the individual has epilepsy Sensitivity of first routine interictal EEG: 30- 60% Problematic false positive rates: paroxysmal activity seen in 30%, frankly epileptiform activity in 5% of normal children Useful in identifying seizure types, seizure syndrome and etiology
62
How is epilepsy diagnosed?
- History (before, during and after event) - Video recording of event - ECG in convulsive seizures (rule out long QT syndrome) - Interictal/ ictal EEG MRI Brain: to determine etiology eg. Brain malformations/ brain damage Genetics: idiopathic epilepsies are mostly familial; also single gene disorders eg. Tuberous sclerosis Metabolic tests: esp if associated with developmental delay/ regression
63
What is the management of epilepsies in children?
Anti-epileptic drugs (AED) should only be considered if diagnosis is clear even if this means delaying treatment Role of AED is to control seizures, not cure the epilepsy - Start with one AED: slow upward titration until side-effects manifest or drug is considered to be inefficient. Age, gender, type of seizures and epilepsy should be considered in selecting AEDs S/Es: CNS related can be detrimental; Drowsiness, effect on learning, cognition and behavioural
64
What drug treatment is available for epilepsy management?
Sodium Valproate (not in girls- recent MHRA advice) or Levetiracetam: first line for generalised epilepsies Carbamazepine: first line for focal epilepsies Several new AEDs with more tolerability and fewer side effects: Levatiracetam, Lamotrigine, Perampanel Other therapies: steroids, immunoglobulins and ketogenic diet (mostly for drug-resistant epilepsies)
65
What are some more management strategies for epilepsy (usually for drug resistant epilepsies)?
VNS (palliative-lessen burden of seizures on every day life) SURGERY (palliative or curative-localise origin & may facilitate a resection)
66
Assessing a child with an unusual head, why is this important?
Accurate measurements & interpretation as part of a childhood physical exam Head size problems=Macrocephaly & microcephaly Head shape problems-What not to miss Refer early for specialist opinion
67
In what order do the fontanelles close?
At birth, the bones of the infant skull are not fused together Posterior fontanelle usually closes 2-3 months after birth Followed by gradual fusion of the sutures Anterior fontanelle usually closes between 1-3 years of age (average=18 months)
68
What measurements should be done in terms of head circumference and in who should they be done?
Occipitofrontal circumference As routine: Between birth- 3 years In any child with neurological symptoms or developmental complaints Measure and plot parental head sizes Too big, too small or odd shape? Sutures & Fontanelles? Look at the facial features- any asymetry?
69
What is microcephaly and what does it usually indicate?
Definition: OFC <2 SD: mild OFC <3 SD: moderate/ severe Microcephaly usually indicates small brain: ‘micranencephaly’ Prenatal or postnatal onset: is it crossing centiles downwards? Timing of onset may be a clue Multiple causes: antenatal, postnatal, genetic and environmental
70
What is MACROcephaly and what should be considered?
Definition: OFC > 2SD Is it crossing centiles upwards?- indicates that either the underlying brain or the underlying CSF is expanding in a way it shouldn't Sutures?-separation Fontanelles?-very large Familial? Hydrocephalus? Large brain? Development normal? Other physical abnormalities – facial features, hepatosplenomegaly, bony deformities etc
71
Head shape problems usually in first year of life: what should be asked and what are the types of head shape problems to be aware of?
Timing of onset? Is it getting better or worse? Plagiocephaly ‘flat-head’ Brachycephaly ‘short head or flat at back’ Scaphocephaly ‘boat shaped skull’ Craniosynostosis (premature fusion of the cranial sutures)
72
What is one of the commonest head shape problems
Deformational plagiocephaly - Due to position child is in - Parallelogram shape skull
73
What is craniosynostosis?
Premature fusion of one or more sutures Depending on which suture closed too early you may get some classical characteristic shapes which may indicate the type of synostosis is
74
How is craniosynostosis usually assessed?
Either cranial CT scans or occasionally skull x-rays
75
What would make you suspect a NM disorder?
- Baby ‘floppy’ from birth - Slips from hands - Paucity of limb movements - Alert, but less motor activity - Delayed motor milestones - Able to walk but frequent falls
76
What does the Gowers sign indicate?
Weakness around the pelvic girdle and lower limb muscles in particular Prominent clinical sign which may indicate an underlying muscle condition (e.g. Muscular dystrophy)
77
What is the most common muscular dystrophy occurring in childhood?
Duchenne muscular dystrophy 1/3500 male infants
78
What gene is affected in DMD and what are the signs of this condition?
Xp21, dystrophin gene, 1/3500 male infants Delayed gross motor skills Symmetrical proximal weakness - Waddling gait, calf hypertrophy - Gower’s sign positive Elevated Creatinine Kinase levels = >1000 in DMD Cardiomyopathy Respiratory involvement in teens
79
Pes cavus (high arch feet) is a classic sign seen in what?
Some types of hereditary motor sensory neuropathies (typified by the charcot marie tooth disease)
80
What is the anatomical approach to neuromuscular conditions to determine where is the sight of the lesion and what is the lesion?
Muscle: muscular dystrophies, myopathies- congenital and inflammatory, myotonic syndromes Neuromuscular junction: myasthenic syndromes Nerve: Hereditary or acquired neuropathies Anterior Horn Cell: Spinal muscular atrophy