Paediatric oncology Flashcards

1
Q

Name 5 oncological emergencies

A
  • Sepsis/febrile neutropenia
  • Raised ICP
  • Spinal cord compression
  • Mediastinal mass
  • Tumour lysis syndrome
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2
Q

Tumor lysis syndrome definition [2]

4 features on blood investigations

A

Metabolic derangement [1] due to rapid death of tumor cells [1]
Hyperphosphatemia, hypocalcemia
Hyperuricemia, hyperuricosuria

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3
Q

Tumor lysis syndrome treatment [6]

A
Hyperhydrate and diuresis
Monitoring: ECG, phosphate
Allopurinol, urate oxidase
Phosphate binders
Correction of hyperkalemia
Dialysis
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4
Q

Treatment of hyperkalemia [3]

A

Calcium gluconate
Salbutamol
Insulin

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5
Q

Febrile neutropenia [2]

A

Neutrophils < 0.5 x 109/L and Fever > 38.0°

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6
Q

Spinal cord compression
Presentation in children [6]
Management [3]

A
Muscle weakness
Spine tenderness
Sphincter disturbance
Sensory disturbance
Back pain
Gait disturbance

Dexamethasone
Chemotherapy
Surgery - debulking, laminectomy, lminotomy, laminoplasty

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7
Q

How to prevent tumour lysis syndrome? [1]

A

Pre-chemotherapy hydration measures

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8
Q

Define purpura

Differentials of purpura [6]

A

Bleeding into skin from small blood vessels to produce a non-blanching rash.

Differentials
• Meningococcal septicaemia
• ALL
• Viral infection e.g. measles, enteroviruses
• Vasculitis e.g. Kawasaki, Henoch Schonlein Purpura
• Idiopathic thrombocytopenic purpura
• Congenital bleeding disorders

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9
Q

Investigations [4]

A

FBC
Blood film
Coagulation screen
Von Willebrands factor screen

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10
Q
Idiopathic thrombocytopenia purpura
Ax [2]
Sxs
Ix [3]
Complication
A

Ax:

  • Type II Hypersensitivity
  • Follows viral infection

Sxs: platelet type bleeding

FBC & blood film
Bone marrow biopsy
CT head

Cx: intracranial haemorrhage

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11
Q

ITP
What would you see on FBC & blood film?
Management [3]

A

FBC & blood film - isolated thrombocytopenia

Mx:

  • Avoid NSAIDs, contact sports
  • Acute: gradual resolution over 3m, supportive tx optional, splenectomy (severe)
  • Chronic: anti-D, RITUXIMAB, splenectomy
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12
Q

Generalized lymphadenopathy

Differentials [5]

A
Viral infection
Bacterial infection
Parasitic: toxo
Autoimmune JIA
Malignancy ALL
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13
Q

Examination of lymphadenopathy. What features of the LN would indicate:
Normal [3]
Malignancy [6]
Infection [4]

A

o Normal: bilateral anterior cervical nodes up to 2cm, axillary nodes up to 1cm, inguinal nodes up to 1.5cm
o Malignancy: supraclavicular (chest or abdo malignancy), epitrochlear (Hodgkin lymphoma), firm, non-tender, matted together or rubbery
o Infection: erythema, warmth, tenderness, fluctuance

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14
Q

Chronic non-specific lymphadenitis
Define [2]
Causative organisms [3]
Ix [2]

A

Chronic inflammation of LN in response to pathogens. Enlarged painless LN

Causative organisms:

  • Cat scratch disease (bartonella hernselae)
  • HSV, toxoplasmosis
  • TB, atypical mycobacterium

Ix: FNAC or biopsy

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15
Q

Chronic non-specific lymphadenitis - Cat scratch disease
Sxs [3]
Mx

A

Fever, headache
Regional lymphadenopathy
Malaise

Mx: conservative

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16
Q

Henoch Schonlein Purpura
Define
Features [6]

A

IgA mediated small vessel vasculitis

Features:

  • Palpable purpuric rash, localized edema
  • Over buttocks and extensor surfaces of arms/legs
  • 1-3d post infection
  • Abdominal pain
  • Polyarthritis
  • IgA nephropathy eg haematuria, renal failure
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17
Q

HSP
Tx [2]
Prognosis [2]

A

Treatment

  • analgesia for arthralgia
  • treatment of nephropathy is generally supportive

Prognosis

  • usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
  • around 1/3rd of patients have a relapse
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18
Q

Purpura NAI

A

Thrombocytopenic bleeding normally on legs or head in crying babies; be suspicious of NAI or severe congenital coagulation disorder if anywhere else

19
Q

Abdominal mass differentials

A

Neuroblastoma

Wilms tumour

20
Q

Neuroblastoma
Epidemiology
Ax [2]
Sxs [6]

A

Ep: 0-5y/o (most common solid tumour in under 5’s)

Ax:

  • embryonal neoplasm derived from sympathetic neuroblasts
  • most commonly adrenal in origin but can be anywhere along sympathetic chain

Sy/Si:

  • abdominal mass
  • lethargy, weight loss
  • hypertension
  • metastasis (lymph nodes, scalp, bone
  • spinal cord compression
  • bruising and swelling of eye
21
Q

Neuroblastoma
Ix [5]
Mx [2]

A

Ix:

  • FBC (thrombocytopenia)
  • raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
  • CT chest abdo pelvis (MRI if suspect spinal lesions)
  • MIBG radioisotope scan (specific to catecholamine)
  • biopsy

Mx:

  • chemotherapy e.g. (CYCLOPHOSPHAMIDE and DOXORUBICIN)
  • RT, surgery
22
Q

Wilms tumour
Ax
RF [3]

A
Ax: nephroblastoma
Risk factors:
- Bethwith-Wiedemann syndrome
- WAGR syndrome
- hemihypertrophy
23
Q

Wilms tumour
Sxs [3]
Ix [5]

A

Sy/Si:

  • abdominal mass
  • painless haematuria, flank pain
  • anorexia, weight loss, hypertension

Ix:
- FBC, U&E, urinalysis, CT chest abdo pelvis, AVOID transcutaneous needle biopsy

24
Q

Wilms tumour
Mx [3]
Prognosis

A

Mx:

  • Nephrectomy
  • Chemotherapy
  • RT if andvanced

Prognosis: 90% 5y survival

25
What is the most common cancers in children
Leukaemia - 33% Brain tumour - 25% but higher mortality (posterior common) Extra-cranial
26
Give examples of extra cranial cancers [6]
``` Lymphoma - Hodgkin's / non-Hodgkin's Neuroblastoma Nephroblastoma (Wilms), Hepatic Retinoblastoma Malignant Bone Germ cell - teratoma secrete ALP ```
27
What age group is most likely to get cancer
Peak at 0-4 = highest rate | Another peak at 18-22
28
What are people with Down's likely to get
Leukaemia
29
What suggests immediate referral to hospital (same day) [2]
Unexplained petechiae | HSM
30
What suggests urgent referral (48 hour) [3]
Repeated attendance with same problem New neuro symptoms Abdominal mass
31
What suggests a referral (2 week) [6]
``` Rest pain, Back pain Unexplained lump Lymphadenopathy Unexplained and persistent symptoms Extreme fatigue, Extreme weight loss Changes in mole ```
32
How does febrile neutropenia present [4]
Fever (sometimes low temp) Rigors Drowsiness Shock - tachy, tachypnoea, hypotension, prolonged cap refil, reduced UO, metabolic acidosis
33
How do you manage febrile neutropenia [5]
``` IV access Broad spectrum antibiotics ABC - oxygen / fluids Inotropes Support of PICU ```
34
What cancer can present with spinal cord compression [3]
Almost all tumours Ewing's sarcoma involving vertebral body Medullablastoma Neuroblastoma + germ cell
35
What causes Superior vena cava syndrome [5]
Tumour pressing on vena cava or medisitunum - Lymphoma - Neuroblastoma - Germ cell - Thrombosis
36
Signs of SVCS
``` Facial, neck and upper thoracic plethora Oedema Cyanosis Distended veins Ill Anxious Reduced GCS ```
37
Signs of superior mediastinum syndrome
``` Dyspnoea especially flat Orthopnoea Tachypnoea Cough Wheeze, Stridor ```
38
How do you investigate SVSC / SMS [3] Mx [6]
CXR CT chest ECHO - Keep upright and calm - Urgent biopsy - FBC / pleural aspirate / GCT marker (germ-cell tumour) - Steroid - Chemotherapy, RT - Thrombolytic therapy
39
Retinoblastoma Ax & inheritance [3] Presentation [5] Ix [3]
Heritable (40%) Autosomal dominant of RB gene on 13q14 Non-inheritable 60% ``` Strabismus Leukocoria (white pupil) Decreased vision Proptosis No red light reflex ``` Ix: - Examination under anaesthesia with maximally dilated pupil - Bidimensional ocular USS & MRI - Blood and tumor sample testing for RB1 gene
40
Retinoblastoma What are 5 interventions? What is the 5y survival rate in UK?
1. Chemotherapy 2. Enucleation - eye removal 3. External beam RT 4. Ophthalmic tract brachytherapy 5. Cryotherapy and Transpupillary thermotherapy (TTT) 5 yr survival 99%
41
Retinoblastoma - what are 3 drugs used in chemotherapy
Chemotherapy: - Carboplatin - Vincristine - Etoposide
42
If heritable retinoblastoma, what follow up is needed? [2]
o MRI surveillance: develop new tumours after dx so 6 monthly for 5y o Parental and sibling screening: counselling and pre-symptomatic mx; must consider germline mosaicism as still a risk of having more children with retinoblastoma
43
What to do when a child with an unexplained enlarged abdominal mass presents to the clinic?
Arrange paediatric review with 48 hours