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Paediatrics > Oncology > Flashcards

Oncology Flashcards

1
Q

What is the most common paediatric malignancy ?

A

Neuroblastoma

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2
Q

What percentage of neuroblastomas arise in the adrenal cortex?

A

40%

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3
Q

Infants with neuroblastoma under 1yr have the worst prognosis - true or false?

A

False

Prognosis for < 1 is good as some show tumour regression

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4
Q

What should you suspect if a child presents with opsoclonus myoclonus syndrome ? (Rapid dancing eye movements)

A

Neuroblastoma

50% of presentations have neuroblastoma

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5
Q

What is the main side effect of ifosfasmide?

A

Nephrotoxicity from tubular damage

Haemorrhagic cystitis

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6
Q

What is the main side effect of cyclophosphamide ?

A

Bone marrow suppression
Risk of malignancy
Haemorrhagic cystitis

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7
Q

What is the main side effect of vincristine?

A

Peripheral neuropathy

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8
Q

What is the main side effect of anthracyclines (eg doxorubicin)?

A

Cardiomyopathy

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9
Q

What is the main side effect of etoposide?

A

Anaphylaxis/hypotension in acute phase
Risk of malignancy
Premature menopause
Infertility in long term

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10
Q

What are the features of Gorlin syndrome ?

A 
Aka nevoid basal cell carcinoma syndrome 
Thousands of skin cancers - BCC 
Recurrent jaw cysts 
Palmoplantar pits 
Calcification of fall cerebri
Macrocephaly 
Hypertelorism

10% medulloblastoma

Sun protection
Avoid X-rays

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11
Q

What is the tumour marker for non Hodgkin lymphoma?

A

LDH

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12
Q

What are the tumour markers for neuroblastoma?

A

HVA (homovanillic acid)
VMA (vanillimandelic acid)
In urine

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13
Q

What is the tumour marker for hepatoblastoma?

A

A fetoprotein

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14
Q

What is the most likely tumour ?
6 yo presenting with headache diplopia facial asymmetry
MRI shows lesion within 4th ventricle
With hypointense calcification

A

Ependymoma

NB calcification of tumour within 4th ventricle is highly suggestive

Mean age = 6 yo
Raised ICP
CN involvement common

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15
Q

What are the histological findings of ependymoma?

A

Perivascular pseudorosettes
Ependymoma rosettes
Monomorphic nuclear morphology

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16
Q

What tumour should you suspect from a cerebellar lesion

Contrast enhancing nodule within the wall of a cystic mass?

A

Pilocytic astrocytoma

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17
Q

What are the histological characteristics of pilocytic astrocytoma?

A

Rosenthal fibres

Bundles of compact fibrillary tissue interspersed with loose microcystic spongy areas

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18
Q

What syndrome is associated with pilocytic astrocytoma ?

A

NF 1

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19
Q

What syndrome is associated with ependymomas?

A

NF 2

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20
Q

What syndrome is associated with choroid plexus papilloma?

A

Von Hippel Lindau

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21
Q

Which chemotherapeutic agent arrests cell cycle in the S phase ?

A

Prednisone

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22
Q

Which stage of the cell cycle does cisplatin affect?

A

Non cell cycle specific

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23
Q

What is the mechanism of action of etoposide?

A

Inhibits topoisomerase II

24
Q

What is the mechanism of action of vincristine?

A

Binds to tubulin inhibiting microtubule formation

Arrests all cells in metaphase

25
Q

What is the mechanism of action of cyclophosphamide ?

A

Metabolite phospharamide mustard crosslinks DNA causing irreversible cell death

26
Q

What is the mechanism of action of doxorubicin?

A

Intercalates DNA thereby inhibiting progression of topoisomerase II (which allows for transcription of DNA)

27
Q

What is the mechanism of action of cisplatin?

A

Crosslinks DNA interfering with mitosis

28
Q

What is the causative organism?
Boy with ALL presenting with neurological signs
India ink stain shows halo around organism

A

Cryptococcus neoformans

Opportunistic fungal infection
Commonest cause of fungal meningitis in immunocompromised host
Halo indicates encapsulated org stain can’t penetrate

29
Q

What is the clinical syndrome of VOD?

A

Tender hepatomegaly with deranged LFTs
Jaundice
Ascites

30
Q

What is the cancer associated with CD33 pos B cell marker?

A

AML

31
Q

What is the cancer associated with CD 30 and CD15 pos B cell marker?

A

Hodgkin lymphoma

32
Q

What is the significance of CD 34 pos cells ?

A

Pluripotent stem cells

33
Q

Which cancer is associated with SMARC1B mutation?

A

Atypical teratoid/rhabdoid tumours

34
Q

Which cancer histopathology - rosenthal fibres ?

A

Pilocytic astrocytoma

35
Q

What is a treatment for astrocytoma ?

A

Tematinib (BRAF mutation)

36
Q

Good or poor prognostic indicators for ALL?

T(12,21) TEL AML

A

Good

37
Q

Good or poor prognostic indicators for ALL?
T(9,22) BCR ABL
T(4,11) MLL AF4

A

Poor

38
Q

Good or poor prognostic indicators for AML?
T(8,21)
T(16,16)
T (15,17)

A

Good

39
Q

Good or poor prognostic indicators for AML?
FLT3
KIT
KRAS

A

Poor

40
Q

What is the treatment for APML?

A

All trans retinoic acid

41
Q

Which types of non Hodgkin lymphoma are CD20 pos?

A

Burkitts
Diffuse large B cell

Treatment Rituximab

42
Q

Can you give raspuricase to someone with G6PD?

A

No

43
Q

What cancer is associated with Horner syndrome?

A

Neuroblastoma

44
Q

What cancer is associated with Hutchinson syndrome ?

A

Neuroblastoma

Bone Mets

45
Q

How do differentiate between neuroblastoma and wilm’s tumour?

A

Neuro - irritable, unwell; calcifications, crosses midline

Wilms- well looking child
Non calcified
Mets to lungs (cannonball Mets)

46
Q

MYCN application is a good or poor prognostic sign for neuroblastoma ?

A

Poor

47
Q

What is Kerner Morrison syndrome ?

A

Intractable secretory diarrhoea die to VIP secretion in neuroblastoma

48
Q

Which syndrome is associated with the highest risk of Wilm’s?
Beckwith Wiederman
Denys drash
WAGR

A

Denys Drash 90%
WAGR 50%
BW 4%

49
Q

Which syndromes are associated with both Wilms and Hepatoblastoma?

A

Beckwith Wiederman
Hemihypertrophy
Simpson Golabi Behmel

50
Q 
What is the syndrome? 
Wilms 
Extra nipple 
Macroglossia 
Hypertelorism
A

Simpson golabi

51
Q

What is the syndrome?
No iris
Wilms

A

WAGR

52
Q

What is the syndrome ?
Ambiguous genitalia
Wilms

A

Denys Drash

53
Q

Which translocation is associated with infant ALL?

A

T(4,11)
MLL AF4
Poor prognosis

MLL gene codes for Histone methyltransferase (epigenetic modifier)

54
Q

What is stage 4S neuroblastoma ?

A

Infants <1 yo
Localised primary with dissemination to skin, liver, bone marrow
Good prognosis

55
Q

How do you differentiate between osteosarcoma and Ewing’s ?

A

Osteo more common; ends of long bones E.g. distal femur, proximal tibia; soft tissue mass with calcification (sunburst pattern)
Ewing’s - neck/shaft of long bones; onion skin appearance on X-ray

Paediatrics (17 decks)

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