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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
What is the mechanism of action of cyclophosphamide ?
Metabolite phospharamide mustard crosslinks DNA causing irreversible cell death
26
What is the mechanism of action of doxorubicin?
Intercalates DNA thereby inhibiting progression of topoisomerase II (which allows for transcription of DNA)
27
What is the mechanism of action of cisplatin?
Crosslinks DNA interfering with mitosis
28
What is the causative organism? Boy with ALL presenting with neurological signs India ink stain shows halo around organism
Cryptococcus neoformans Opportunistic fungal infection Commonest cause of fungal meningitis in immunocompromised host Halo indicates encapsulated org stain can’t penetrate
29
What is the clinical syndrome of VOD?
Tender hepatomegaly with deranged LFTs Jaundice Ascites
30
What is the cancer associated with CD33 pos B cell marker?
AML
31
What is the cancer associated with CD 30 and CD15 pos B cell marker?
Hodgkin lymphoma
32
What is the significance of CD 34 pos cells ?
Pluripotent stem cells
33
Which cancer is associated with SMARC1B mutation?
Atypical teratoid/rhabdoid tumours
34
Which cancer histopathology - rosenthal fibres ?
Pilocytic astrocytoma
35
What is a treatment for astrocytoma ?
Tematinib (BRAF mutation)
36
Good or poor prognostic indicators for ALL? | T(12,21) TEL AML
Good
37
Good or poor prognostic indicators for ALL? T(9,22) BCR ABL T(4,11) MLL AF4
Poor
38
Good or poor prognostic indicators for AML? T(8,21) T(16,16) T (15,17)
Good
39
Good or poor prognostic indicators for AML? FLT3 KIT KRAS
Poor
40
What is the treatment for APML?
All trans retinoic acid
41
Which types of non Hodgkin lymphoma are CD20 pos?
Burkitts Diffuse large B cell Treatment Rituximab
42
Can you give raspuricase to someone with G6PD?
No
43
What cancer is associated with Horner syndrome?
Neuroblastoma
44
What cancer is associated with Hutchinson syndrome ?
Neuroblastoma | Bone Mets
45
How do differentiate between neuroblastoma and wilm’s tumour?
Neuro - irritable, unwell; calcifications, crosses midline Wilms- well looking child Non calcified Mets to lungs (cannonball Mets)
46
MYCN application is a good or poor prognostic sign for neuroblastoma ?
Poor
47
What is Kerner Morrison syndrome ?
Intractable secretory diarrhoea die to VIP secretion in neuroblastoma
48
Which syndrome is associated with the highest risk of Wilm’s? Beckwith Wiederman Denys drash WAGR
Denys Drash 90% WAGR 50% BW 4%
49
Which syndromes are associated with both Wilms and Hepatoblastoma?
Beckwith Wiederman Hemihypertrophy Simpson Golabi Behmel
50
``` What is the syndrome? Wilms Extra nipple Macroglossia Hypertelorism ```
Simpson golabi
51
What is the syndrome? No iris Wilms
WAGR
52
What is the syndrome ? Ambiguous genitalia Wilms
Denys Drash
53
Which translocation is associated with infant ALL?
T(4,11) MLL AF4 Poor prognosis MLL gene codes for Histone methyltransferase (epigenetic modifier)
54
What is stage 4S neuroblastoma ?
Infants <1 yo Localised primary with dissemination to skin, liver, bone marrow Good prognosis
55
How do you differentiate between osteosarcoma and Ewing’s ?
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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