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Paediatrics > Oncology > Flashcards

Oncology Flashcards

1
Q

ewing sarcoma prevalence

A

second most common primary bone cancer in children
rare <100 cases diagnosed in UK
more commonly affects male
10-20’s

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2
Q

ewing sarcoma pathophysiology

A

small, round
blue cell tumour
in majority of cases - genetic mutation - translocation between chromosome 11 and 22 resulting in formation of a fusion gene which does for fusion protein. This is a transcription factor that upregulates cell proliferation…uncontrolled cell turnover

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3
Q

ewing sarcoma bonesc

A

flat bones - tibia, fibula, femur, pelvis, ribs

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4
Q

clinical features ewing sarcoma

A

misinterpreted as growing pains or sports injuries
unexplained bony lump - urgent x ray within 48 hrs

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5
Q

x ray ewing sarcoma

A

urgent referral for an appt within 48 hrs for specialist assessment

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6
Q

hx ewing sarcoma

A

bone pain - progressive, worse at night, OTC analgesia not work
restricted ROM
fatigue
weight loss

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7
Q

examination ewing sarcoma

A

Tender palpable mass
Fever
Increased susceptibility to fracture – this can often be how Ewing sarcoma is diagnosed

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8
Q

ddx ewing sarcoma

A

Tendonitis
Osgood-Schlatter disease
Trauma
Slipped epiphysis

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9
Q

investigations ewing sarcoma

A

FBC, UE, LFT, ESR, CRP, ALP, bone profile - all elevated, anaemia, leucocytosis
X ray - destructive, diaphyseal lesion with layered periosteal calcification
MRI/CT - staging
bone biopsy - definitive

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10
Q

management ewing sarcoma

A

combination of chemo, surgery and radio
chemo - neoadjuvant and adjuvant, VDC/IE regime
surgery - limb sparing with resection and metal implant or autologous bone graft, may require amputation
radio - not every pt, before surgery to shrink tumour

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11
Q

poor prognostic factors ewing sarcoms

A

Metastatic disease at diagnosis
Large tumour size (≥200ml in volume or ≥8cm in diameter)
Primary tumour located in the axial skeleton, especially the pelvis
Histological response of less than 100%

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12
Q

complications ewing sarcoma

A

metastatic disease - lungs, other bones and bone marrow
recurs later in life at around 30% of individuals
60% live at least 5 yrs

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13
Q

nephroblastoma definition

A

aka wilm’s tumour
most common renal tumour affecting children

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14
Q

epidemiology nephroblastoma

A

80 children per year in UK
pre school age group
female more

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15
Q

pathophysiology nephroblastoma

A

arise from nephrogenic rests - embryonal remnants
also mutations of common tumour suppressor genes

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16
Q

risk factors nephroblastoma

A

WAGR (Wilm’s tumour, Aniridia, Genitourinary malformations, and Retardation), Denys-Drash and Beckwith-Wiedemann

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17
Q

hx nephroblastoma

A

abdo mass/swelling
pain
fever
haematuria

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18
Q

exam nephroblastoma

A

abdo distention with unilateral or b/; mass

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19
Q

ddx nephroblastoma

A

PCKD
hydronephrosis
neuroblastoma

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20
Q

investigations nephroblastoma

A

FBC, UE, urine dip
USS
CT/MRI
biopsy - defintiive
risk scoring (1-5)

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21
Q

management nephroblastoma

A

healthy BP
avoid contact sports
stage 1 and 2 - just surgery
chemo before surgery
surgery - nephrectomy

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22
Q

prognosis nephroblastoma

A

even with mets 85% of pts expected to be cured
effects of chemo - cardiotoxicity

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23
Q

neuroblastoma origins

A

derived from neural crest cells - typically arising from adrenal glands or abdominal sympathetic chain

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24
Q

neuroblastoma epidemiology

A

90 children per year in UK
most common solid tumour in children

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25
Q

neuroblastoma pathophysiology

A

Like most cancers of infancy, neuroblastoma arises from poorly differentiating embryonic cells (blasts), in this case from the neural crest. Neural crest cells are derived from developing ectoderm, and normally migrate throughout the body to form a range of structures including the sympathetic nervous system and adrenal medulla. When this migration is stalled, neural crest cells have the potential to acquire mutations that eventually lead to a neuroblastoma.

Whilst familial cases of neuroblastoma are rare, neuroblastoma tumours are associated with a specific profile of acquired genetic mutations, in particular of the MYCN and ALK oncogenes, as well as loss-of-function of the tumour suppressor PHOX2B

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26
Q

risk factors neuroblastoma

A

It’s incidence is therefore more likely if the child has other neurocristopathies, such as Hirschsprung’s Disease or Congenital Central Hypoventilation Syndrome

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27
Q

hx neuroblastoma

A

abdo distention
fatigue
weight loss
creased catecholamine secretion (sweating, agitation) or metastasis (bone pain that prevents sleep, recurrent infections). Compression of the sympathetic nervous system can occasionally lead to urinary incontinenc
arise from thoracic portion - SOB, CP

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28
Q

examination neuroblastoma

A

dense abdo swelling
HTN and tachycardia
periorbital bruising - mets to skull base
bluberry muffin rash
recurrent infection
thrombocytopenic purpura

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29
Q

ddx neuroblastoma

A

cysts (hepatic, polycystic kidney disease), hyperplasia (pyloric stenosis, hepatomegaly, splenomegaly) or neoplasia (Wilms’ tumour, lymphoma, rhabdomyosarcoma, hepatoblastoma).

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30
Q

investigations neuroblastoma

A

the products of catecholamine breakdown - homovanilic acid and vanillymandelic acid in urine - both raised
bone marrow and skin biopsies
USS
MRI
CXR if thoracic
MIBG scan - definitive test

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31
Q

staging neuroblastoma

A

international neuroblastoma risk group (INRG)
1, 2A, 2B, 3, 4, 4S

32
Q

management neuroblastoma

A

if <18mths old - likely to regress to nothing or benign ganglioma
older children - surgery is preferred, L1 - curative, if L2 - adjuvant chemo or radio
novel immunotherapies
clinical trials - proto-oncogene inhibitor
anti-emetics
analgesia

33
Q

prognosis neuroblastoma

A

5 year survival rate is 67%
if diagnosed before 1 year of age - 82%
1-5 yrs old - 43%

34
Q

good prognostic factors neuroblastoma

A

younger age at diagnosis
female
lesser tumour stage at diagnosis
MYCN mutation absent

35
Q

complication neuroblastoma

A

relapse which is often resistant to tx
OMA syndrome - autoimmune condition, leading to opsoclonus, myoclonus, ataxia, confusion and irritability

36
Q

SVC syndrome definition and causes

A

results from compression or obstruction of major vessels
life-threatening
most common causes - anterior mediastinal masses secondary to lymphomas and leukaemias, also from thrombosis

37
Q

SVC syndrome presentation

A

reduced venous return from head and neck to heart
airway compression - reduced TLC

  • face and neck oedema
    distention of neck and thoracic veins
    plethora
    headaches
    dizziness
  • dyspnoea
    hoarse voice
    cough
    oropharyngeal obstruction
    orthopnoea
38
Q

SVC syndrome investigations

A

CXR - mediastinal widening, pleural effusion
CT chest
echo - pericardial effusion
bloods - FBC, UE, blood film, LDH

39
Q

SVC syndrome management

A

A-E assesment
haem and onc consultants
avoid giving fluid via cannula in upper limbs

40
Q

spinal cord compression definition

A

can lead to permanent loss of sensory, motor and/or autonomic function
acute complication of mets - undiagnosed cancer

41
Q

SSC causes

A

Tumour extension through the vertebral foramina into the epidural space (most common)
Intradural metastases
Crush fractures from bony vertebral metastases or extended steroid use

42
Q

investigation SSC

A

MRI spine

43
Q

presentation SSC

A

Unexplained, persistent back pain which wakes the child from sleep, restricts movement and/or does not resolve with simple analgesia
Limb weakness/paraesthesia
Progressive weakness below the level of the suspected lesion
Bladder dysfunction/incontinence
Constipation or overflow diarrhoea

44
Q

management SSC

A

surgical - NBM, catheter, cord decompression
medical - dexamethasone, analgesia, laxatives

45
Q

tumour lysis syndrome definition

A

overwhelming cell lysis…mass release of intracellular cell content into circulation..metabolic abnormalities
if suffer with acute lymphoblastic leukaemia, non-hodgkin lymphoma or high proliferative malignancies - high risk of TLS

46
Q

tumour lysis syndrome presentation

A

hyperuricaemia - Oliguria or anuria, crystal formation in urine, hypertension, renal insufficiency
hyperkalaemia - nausea, slow/irregular pulse, muscle weakness, cardiac arrythmias
hyperphosphataemia - not obvious
hypercalcaemia - Muscle cramps, facial twitch, paraesthesia, seizures, ECG abnormalities

47
Q

investigations TLS

A

bloods - FBC, UE, bone profile, urate
fluid balance
daily weight

48
Q

management TLS

A

prevention - allopurinol
treatment - hyperhydration so high urine output, rasburicase, dialysis

49
Q

febrile neutropenia define

A

Fever defined as a single temperature of on one occasion. Neutropenia is characterised as an absolute neutrophil count of <0.5 x109/L9

50
Q

hx febrile neuropenia

A

recent blood tests
recent chemo
myelosuppression
shaking chills, rigor
previous isolation of gram neg bacteria
recent tx with antimicrobial therapy

51
Q

febrile neutropenia exam

A

LOOK FOR SOURCE
Oropharynx – consider mucositis, hepatic stomatitis, candidiasis
ENT – take viral nasopharyngeal and throat swabs for extended viral screen.
Upper gastrointestinal – painful swallowing may suggest herpetic or candidal oesophagitis.
Abdominal – signs of colitis or typhlitis (caecal inflammation causing tenderness of the right lower quadrant)
Perineum – always ask about perianal discomfort
Central venous line sites – examine for erythema, swelling, tenderness or tracking along the line site
Skin exam – look for rashes, examine bone marrow aspirate sites

52
Q

investigations febrile neutropenia

A

Bloods – FBC, U+Es, CRP, venous blood gas, lactate
Blood cultures
Urine/Stool culture
Sputum culture
Imaging – chest and/or abdominal x-ray or CT
Swab – nasal/throat/ski

53
Q

febrile neutropenia management

A

empirical abx within 1hr of arrival - tazocin +/- gentamicin +/- teicoplanin
repeat cultures

54
Q

osteosarcoma prevalence

A

most common childhood primary bone cancer
in teens and young adults and adults >50
more common in males
and afro-caribbean

55
Q

osteosarcoma pathophysiology

A

DNA mutations occur in rapidly diving osteoblasts leading to malignant transformation
- metaphysis of long bones

56
Q

osteosarcoma types

A

steoblastic, chondroblastic and fibroblastic
The osteoblastic subtype arises from the most highly differentiated cells, the fibroblastic subtype arises from the least differentiated cells, and the chondroblastic subtype is somewhere in between

57
Q

risk factors osteosarcoma

A

Li-Fraumeni syndrome- an autosomal dominant germline mutation affecting the p53 protein (a tumour suppressor protein).
RB1 mutation- this affects the retinoblastoma protein, causing hereditary retinoblastoma. Osteosarcoma is the most common tumour caused by this mutation, aside from retinoblastoma itself.
Other genetic conditions include Rothmund-Thomson Syndrome, Bloom Syndrome, Werner Syndrom

58
Q

clinical features osteosarcoma

A

pain at tumour site
intermittent, worsens at night, resistant to analgesia
lump, warm and tender
mobility issues
fatigue, weight loss, headaches

59
Q

ddx osteosarcoma

A

Secondary bone tumours (this is more likely in adults than in children)
Benign bone tumours
Infection (e.g. osteomyelitis)

60
Q

investigations osteosarcoma

A

X ray - Bone destruction
New bone formation
Periosteal swelling
Soft tissue swelling
definitive - biopsy of affected area

61
Q

management sarcoma

A

surgery and chemo (doxorubicin, cisplatin and high dose methotrexate +/- mifamurtide)
amputation may be required

62
Q

prognosis osteosarcoma

A

poor
5 year survival rate 60%
as late diagnosis and aggressive

63
Q

complications osteosarcoma

A

pathological fractures
mets

64
Q

CNS tumour

A

primary - originate from brain
secondary - spread from other parts

65
Q

brain tumours epidemiology

A

400 young people per year in UK
10 diagnosed per week
boys more

66
Q

most common groups of CNS tumours

A

Astrocytoma (low and high grade gliomas that develop from glial cells) [40%]
Medulloblastoma (usually develop in the posterior fossa/cerebellum) [13%]
Ependymoma (formed from cerebrospinal fluid (CSF) producing ependymal cells) [7%]
Craniopharyngioma (found at the base of the brain close to the pituitary gland) [5%]
Germ cell tumours (arising from germ cells, usually found close to the pituitary gland and the pineal gland) [4%]
Choroid plexus tumours (develop from network of ependymal cells) [2%]

67
Q

risk factors CNS tumours

A

Personal or family history of a brain tumour, leukaemia, sarcoma, and early onset breast cancer
Prior therapeutic CNS irradiation
Neurofibromatosis 1 and 2
Tuberous sclerosis 1 and 2
Other familial genetic syndromes (such as von Hippel-Lindau)

68
Q

clinical features CNS tumour

A

delayed milestones
neurodevelopmental delay
differential education attainment
headache
polyuria/dipsia
seizures
altered GCS

69
Q

examination CNS tumours

A

General examination: Child’s behaviour, consciousness level and alertness
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

70
Q

ddx CNS tumours

A

migraine or tension headache
meningitis
IC haemorrhage/stroke

71
Q

investigations CNS tumours

A

MRI first choice

72
Q

management CNS tumours

A

analgesia, antiemetics, anticonvulsants, fluid/diet, steroids
surgical resection
radiotherapy
cehmo
proton therapy or stem cell transplants
fetility support and neuro-rehab

73
Q

complications CNS tumours

A

epilepsy and seizures
sleep disturbance
effects on puberty.fertility
hearing loss
impaired growth
cognitive impairment
secondary malignancy

74
Q

prognosis CNS tumours

A

The 5-year survival in England for all Brain and spinal tumours in children is 73%
Low grade astrocytoma has a survival rate of about 95%
High grade (anaplastic) astrocytoma has a survival rate of about 25%
Ependymoma has a survival rate of about 80%
Medulloblastoma, it is more than 60%

75
Q
A

Paediatrics (17 decks)

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