Oncology Flashcards

1
Q

What is a wilms tumour (nephroblastoma)

A

mc renal tumour of childhood originating from embryonal renal tissue

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2
Q

At what age does a wilms tumour typically present

A

Before the age of 5

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3
Q

Give clinical features of a wilms tumour

A
  • unilateral large abdominal mass
  • painless haematuria
  • Anaemia
  • flank pain
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4
Q

How is a wilms tumour diagnosed

A
  • Ultrasound and/or CT/MRI of abdomen - shows intrinsic renal mass
  • children with an unexplained enlarged abdo mass should have a paeds review within 48hrs
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5
Q

How is a wilms tumour managed

A
  • Initial chemo followed by delayed nephrectomy
  • Staged histologicially then subsequent Tx is planned according to findings
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6
Q

What is a retinoblastoma

A

Malignant tumour of retinal cells
that can be bilateral or unilateral

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7
Q

What does a bilateral retinoblastoma indicate about the cause

A

Hereditary

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8
Q

What chromosome is the retinoblastoma susceptibility gene found and what is the pattern of inheritance

A
  • chromosome 13
  • Dominant inheritance pattern with incomplete penetrance
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9
Q

At what age do children typically present with retinoblastomas

A

first 3 years of life

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10
Q

Give 2 clinical features of a retinoblastoma

A
  • white pupillary reflex (replaces normal red one)
  • Squint
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11
Q

How is a retinoblastoma diagnosed

A
  • ophthalmic A- and B-scan ultrasound
  • fundoscopy and exam under anaesthesia
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12
Q

How is a retinoblastoma treated

A
  • Aims to cure and preserve vision
  • Chemotherapy to shrink tumour followed by local laser treatment
  • Enucleation in advanced disease
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13
Q

What are the two most common leukaemias in children

A
  • Acute lymphoblastic leukaemia (ALL) - most common
  • Acute myeloid leukaemia (AML) - 2nd most common
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14
Q

Give 3 RFs for leukaemia in children

A
  • Down’s syndrome
  • Kleinfelter syndrome
  • Noonan syndrome
  • radiation exposure during pregnancy
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15
Q

Describe how leukaemia may present in children

A
  • anaemia - lethargy and pallor
  • neutropenia - frequent or severe infections
  • thrombocytopenia - easy bruising, petechiae
  • failure to thrive
  • fever
  • hepatosplenomegaly
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16
Q

How is leukaemia investigated in children

A
  • NICE recommend referring any child with unexplained petechiae or hepatomegaly for specialist assessment
  • FBC within 48h if leukaemia is suspected
  • Bone marrow biopsy is diagnostic
17
Q

Poor prognostic factors of acute lymphoblastic leukaemia

A

age < 2 years or > 10 years
WBC > 20 * 109/l at diagnosis
T or B cell surface markers
non-Caucasian
male sex

18
Q

Give 5 types of brain tumour seen in children

A
  • astrocytoma
  • medulloblastoma
  • ependymoma - Commonly seen in the 4th ventricle
  • brainstem glioma
  • craniopharyngioma - solid/ cystic tumour derived from embryological remnants of rathke’s pouch
19
Q

What is a medulloblastoma

A

aggressive paediatric brain tumour that arises within the infratentorial compartment and spreads through the CSF

20
Q

What is the most common primary brain tumour in children

A

Pilocytic astrocytoma

21
Q

How may a brain tumour present in children

A
  • raised intracranial pressure - Headache worse on waking, coughing, straining or bending forward
  • Back pain
  • Peripheral weakness of arms/legs
  • Bladder/bowel dysfunction
22
Q

How is a brain tumour managed

A
  • diagnosed on MRI scan
  • surgery - aimed at treating hydrocephalus
  • chemo/ radiotherapy depending on tumour site and patient age
23
Q

What is a neuroblastoma

A

tumour arising from neural crest tissue of the adrenal medulla (mc site) and sympathetic nervous system

24
Q

Describe how a neuroblastoma may present in children

A
  • abdominal mass
  • pallor
  • weight loss
  • bone pain, limp
  • hepatomegaly
  • periorbital bruising and buldging
25
Q

How is a neuroblastoma investigated

A
  • raised catecholamine levels (urinary vanillylmandelic acid and homovanillic acid)
  • biopsy
  • abdo XR may show calcification
26
Q

Give two types of bone cancers seen in children

A
  • osteosarcoma
  • ewing’s sarcoma
27
Q

How may bone cancers present in children

A
  • affecting pelvis and long bones in ewing’s
  • affecting long bones prior to epiphyseal closure in osteosarcoma
  • persistent bone pain, worse at night
  • bone swelling
28
Q

How are bone cancers investigated in children

A
  • XR within 48h
  • osteosarcoma: destruction and variable periosteal new bone formation and sunburst pattern
  • ewing’s: soft tissue mass - onion skin appearance
29
Q

How are bone cancers managed in children

A
  • combination chemo before surgery
  • surgical resection of lesions often with amputation
  • MDT approach
30
Q

How does a hepatoblastoma present in children

A
  • abdo distension/ mass
  • abdo pain
  • jaundice (rare)
31
Q

What tumour marker is nearly always raised in hepatoblastomas

A

alpha fetoprotein