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Oncology > Oncology LP CSV > Flashcards

Oncology LP CSV Flashcards

1
Q

What cancers are associated with RB germ line mutation?

A

retinoblastoma, osteosarcoma, pineoblastoma, melanoma

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2
Q

what brain cancers do you use chemotherapy?

A

germinoma, medulloblastoma, ependymoma, low grade gliomas

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3
Q

What are the effects of cranial irradiation?

A

neurocognitive injury (most common), endocrinopathies (GH>FSH/LH/TSH), neurovascular disease (e.g. moyamoya)

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4
Q

what is the classic triad of brain tumour and which type of tumours most likely present this way?

A

Triad: headache, nausea & vomiting and papiloedema.

Infratentorial tumours more likely present this way - gliomas, craniopharyngiomas, medulloblastoma

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5
Q

What can torticollis result in?

A

cerebellar tonsil herniation

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6
Q

How do supratentorial tumours more commonly present? Give an example of a supratentorial tumour.

A

focal weakness, sensory impairment, seizures, speech or reflex abnormalities.
Example astrocytomas, ependymomas

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7
Q

A child presents with diencephalic syndrome? what symptoms will they have and where might the tumour be?

A

diencephalic syndrome = FTT, emaciation, increased appetite, euphoria and increased activity.
Seen with supratentorial tumours & 3rd ventricle. Most common glioma

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8
Q

what is Parinaud syndrome and what tumour is it associated with?

A

inability to look up, pupillary dilatation (not reactive to light), nystagmus, eyelid retraction. associated with pineal region tumours - PNET, glioma

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9
Q

What are the posterior fossa tumours? What ages do they occur?

A 
medulloblastoma (young children); 
pilocytic astrocytoma (older children), ependymoma
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10
Q

What are the inheritance syndromes for brain tumours? what is their inheritance?

A

NF-1 (glioma), NF2 (schwannoma), TS (astrocytoma), VonHippel Lindau (haemangioblastoma), Li-Fraumeni (astrocytoma), Cowden (gangliocytoma), Turcot (medulloblastoma) ALL AUTOSOMAL DOMINANT INHERITANCE

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11
Q

What are the most common brain tumours in 0-14 year olds?

A

pilocytic astrocytoma, medulloblastoma

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12
Q

what are the most common brain tumours in 15-19year olds?

A

pilocytic astrocytoma, pituitary tumours

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13
Q

what syndrome are pilocytic astrocytomas associated with? How many become malignant?

A

NF-1, low grade gliomas,

<5% malignant transformation

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14
Q

Which glioma can be diagnosed on imaging alone?

A

diffuse intrinsic pontine glioma. incurable.

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15
Q

What are the features of medulloblastoma?

A

occur in cerebellum only,
small round blue cell tumour,
aggressive - evolve over weeks to months, malignant

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16
Q

What deletion is commonly associated with medulloblastoma?

A

Cr 17p deletion (associated 30-40% cases)

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17
Q

What is favourable prognostic markers for medulloblastoma?

A

sonic hedgehog
WNT
TRKC

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18
Q

what is unfavourable prognostic indicator for medulloblastoma?

A

MYC amplification

tyrosine kinase receptor ERBB2 amplification

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19
Q

what are the features of ependymoma?

A

grow out of ventricle (usually 4th ventricle), associated with NF-2

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20
Q

What are the midline tumours?

A

glioma (optic tract),
hamartoma (hypothalamus),
germinoma (pituitary stalk/pineal), craniopharynioma (pituitary)

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21
Q

where do craniopharyngiomas originate?

A

Rathke’s pouch

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22
Q

how do craniopharyngiomas present?

A

same as radiation - GH deficiency, FSH/LH deficiency, Hypothyroid.
No problems with prolactin and DI as no stalk involvement

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23
Q

what are the post operative problems after removal of craniopharnygioma?

A

Diabetes insipidus, SIADH (secondary to cutting pituitary stalk)

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24
Q

How can a craniopharyngioma present on imaging?

A

can be a cystic structure with calcifications

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25
Q

what adenomas are common in (1) young children and (2) older children?

A

(1) ACTH secreting (2) prolactinoma

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26
Q

What are the clinical features of pituitary stalk germinoma?

A

Peak 10-12years of age,
present with DI, visual changes, headache secondary to hydocephalus, there is pituitary stalk thickening,
CSF will be positive for bHCG & AFP.
can be diagnosed without biopsy

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27
Q

what are the clinical features of hamartomas?

A

girls 2-4 years of age,
gelastic seizures,
precocious puberty

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28
Q

How do you diagnose neuroblastoma?

A

urine catecholamines HVA, VMA & dopamine

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29
Q

what age group is neuroblastoma present?

A

young children <5 years

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30
Q

how does neuroblastoma present?

A

SICK children
abdominal mass,
Can have Horners syndrome, SVC syndrome or spinal cord compression.

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31
Q

what is associated with poor prognosis in Neuroblastoma?

A

N-MyC amplification (1p deletion)

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32
Q

what are the paraneoplastic syndromes associated with neuroblastoma?

A

(1) opsoclonus-myoclonus ataxia (multi-directional eye movements, ataxia. differential disgnosis hepatoblastoma);
(2) watery diarrhoea (VIP secretion);
(3) hypertension

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33
Q

What is characteristic of neuroblastoma metastatic skin lesions?

A

rubbing them induces central blanching and surrounding halo of erythema (lasts 30-60 min)

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34
Q

what are the stages of neuroblastoma?

A

stage 1 = localised;
stage 2a= unilateral tumour with unilateral LN; stage 2b = unilateral tumour LN bilaterally; stage 3 = tumour across midline ;
stage 4 = metastatic spread;
stage 4S = <1year old, localised tumour with dissemination to liver, skin and bone only

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35
Q

what is good prognosis for neuroblastoma?

A

<1year old,
tumour not crossing midline,
hyper diploid

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36
Q

hodgkin lymphoma - age, sex, presentation

A

teens,
females,
prolonged presentation with lymph nodes, mediatinal mass,
B symptoms

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37
Q

Hodgkin lymphoma - cells?

A

Reed Sternburg cells

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38
Q

NHL types

A

2 B - Burkitt & Diffuse large B cell;

2 T - lymphoblastic lymphoma, anaplastic large cell lymphoma

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39
Q

Peak age for ALL

A

2-5 years of age

40
Q

Good prognostic indicators ALL

A 
1-10years old, 
WCC 50 chromosomes), 
Trisomy 4, 10, 17, 
Hyperdiploid
TEL AML, 
Translocation 12:21 (ETV-RUNX) 
no extramedullary disease
41
Q

Bad prognostic indicators ALL

A 
10 years old, 
WCC >50, 
hypodiploid (<44 Chr), 
Philadelphia Chr 9:22 BRC-ABL, 
MLL rearrangement, 
extra medullary disease
42
Q

Sanctuary sites for ALL

A

Testes and brain = worse prognosis

43
Q

What has biggest effect of ALL prognosis

A

Minimal residual disease at the end of induction (>0.01% = bad)

44
Q

T Cell ALL Philadelphia chromosome

A

BRC ABL1 (9,22)

45
Q

Treatment Phladelphia chromosome

A

Tyrosine kinase inhibitiion (IMATINIB) - works by preventing addition of phosphate and switching “off” tyrosine kinase enzyme leading to apoptosis.

46
Q

what other neural crest disorders are associated with neuroblastoma?

A

Hirschsprungs

Congenital hypoventilation syndrome

47
Q

How does a Wilms tumour present?

A

young child that is WELL with abdominal mass

48
Q

what are the risk factors for wilms tumour

A 
beckwith weidemann, 
hemihypertrophy, 
urogenital malformations, 
NF-1, 
WT-1 (DenysDrash, WAGR)
49
Q

what are the favourable factors for rhabdomyosarcoma

A

age 1-10,
<5cm size,
not in bladder/prostate (that is bad)

50
Q

what is syndromes are associated with hepatoblastoma

A 
beckwith weidemann, 
hemihypertrophy, 
FAP, 
Low Birth Weight 
Fetal alcohol syndrome
51
Q

Which lobe is commonly affected in hepatoblastoma

A

right lobe

52
Q

what might be raised in hepatoblastoma

A

AFP, bHCG

53
Q

what syndromes are associated with germ cell tumours

A 
gonadal dysgenesis, 
Klinefelters, 
Turners, 
saccral agenesis, 
Downs and 
russel silver
54
Q

with testicular germ cell tumours what is the effect on fertility

A

prior to treatment usually there is 30% azoospermia and 50% impaired sperm motility already. With treatment this declines further. However there is usually 70% recovery 3 years post treatment.

55
Q

which tumour is seen most commonly secondary to radiation

A

angiosarcoma

56
Q

what tumour are plexiform fibromas in NF-1 linked to ?

A

malignant peripheral nerve sheath tumours.

57
Q

what are the features in osteosarcomas

A

older children,
local pain and swelling,
metaphysis of long bones (distal femur and proximal tibia, proximal humerus),
sunburst pattern on X-ray

58
Q

what are the features of ewings

A

younger children,
systemic symptoms,
diaphysis of long bones and flat bones, translocation 11;22 in 85%

59
Q

what are a feature of an osteoid Osteoma

A

pain is relieved by aspirin

60
Q

what does a osteochondroma look like

A

cauliflower/stalk like appearance found near joints

61
Q

what are the pathological findings specific for langerhan histiocytosis

A

birbeck granules, CD1a and CD207

62
Q

how does multifocal langerhan histiocytosis present

A

mutliple bony lesions, DI, exopthalmos

63
Q

what are the risk organs for langerhan histiocytosis

A

liver, spleen, bone marrow and lungs

64
Q

what defect is linked to x-linked lymphoproliferative disease

A

SAP defect

65
Q

what is the best treatment for lymph proliferative disease

A

Rituximab (antiCD20) and bone marrow transplant

66
Q

what are the diagnostic criteria for HLH

A 
fever >38.5, 
splenomegaly, 
cytopenia (2-3 cell lines), 
haemophagocytosis, 
high triglycerides, 
low fibrinogen, 
ferritin >500, 
raised CD 25, 
low or absent NK activity
67
Q

which AML is best treated with retinoic acid

A

APML (M3)

68
Q

what leukaemia are Down syndrome children at risk of

A

younger than 3 AML >ALL,
older than 3 ALL >AML,
if they have transient myeloproliferative disorder then there is a 20-30% risk of megakaryocytic AML

69
Q

what medication is used in CML

A

Imatinib - inhibits BCR-ABL tyrosine kinase

70
Q

what is the order for best source of haemopoetic stem cell transplant

A

cord blood> bone marrow > peripheral blood

71
Q

what is the order for best donor for haemopoetic stem cell transplant

A

HLA sibling > HLA unrelated> Relative NON-HLA matched

72
Q

if an A patient receives donor graft from an O patient what should you remove

A

plasma

73
Q

what should you remove if the patient is O and the donor is A

A

red blood cells

74
Q

who are the best donors and why

A

males as they have no anti-Y antibodies

75
Q

what cancer does SVC obstruction more likely to occur

A

T cell ALL or lymphoma

76
Q

what is tumour lysis syndrome the highest risk

A

12-72 hrs post starting chemo but risk remains to 7 days

77
Q

how does allopurinol work

A

inhibits xanthine oxidase (can get build up of xanthine which precipitates at pH >7.5)

78
Q

how does rasburicase work

A

recombinant urate oxidase changes uric acid to allantoin

79
Q

what must you never give with rasburicase

A

sodium bicarb

80
Q

what are the indications for dialysis in tumour lysis syndrome

A

phosphate >10, potassium >6, volume overload, low calcium with symptoms

81
Q

what is infection are you particularly at risk of with prolonged neutropenia

A

fungal infection >5-7days

82
Q

what antibiotics should you give for febrile neutropenia

A

amikacin / gent or Tazocin

83
Q

which cancer has the highest rate of secondary malignancies

A

hodgkins lymphoma 7%

84
Q

what si the risk of secondary malignancy with ALL and what cancers are seen?

A

2-3%,

brain tumour and AML seen

85
Q

CYCLOPHOSPHAMIDE

A

Alkylating agent
haemorrhagic cystitis
bladder cancer
infertility

86
Q

VINCRISTINE

A 
acts on microtubules, M phase 
peripheral neuropathy
jaw pain
constipation
burn on extravasation
doesn't cause myelosuppression
87
Q

ASPARGINASE

A

G1 phase

thrombus and DIC

88
Q

DOXORUBICIN

A

anthracycline, S phase
cardiac effects
red urine

89
Q

ETOPOSIDE

A

M phase
secondary malignancy
anaphylaxis
myelosuppression

90
Q

CISPLATIN

A 
all phases, platin
TOXIC 
myelosuppression
hearing loss 
low Mg, K 
hair loss
delayed emesis
91
Q

CYCLOSPORIN

A 
Calcineurin inhibitor 
gingival hyperplasia 
renal toxicity
high BP
high Lipids 
Hirsuitism
92
Q

THIGUAMINE

A

S phase DNA analogue

Veno-occlusive disease

93
Q

CYTOSINE ARABINOSIDE

A

cerebellar toxicity - ataxia, intention tremour

94
Q

IMATINIB

A

QT prolongation

95
Q

CYTARABINE

A

S phase
cytokine storm
strep infection
myelosuppression

Oncology (1 decks)

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