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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
what adenomas are common in (1) young children and (2) older children?
(1) ACTH secreting (2) prolactinoma
26
What are the clinical features of pituitary stalk germinoma?
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
27
what are the clinical features of hamartomas?
girls 2-4 years of age, gelastic seizures, precocious puberty
28
How do you diagnose neuroblastoma?
urine catecholamines HVA, VMA & dopamine
29
what age group is neuroblastoma present?
young children <5 years
30
how does neuroblastoma present?
SICK children abdominal mass, Can have Horners syndrome, SVC syndrome or spinal cord compression.
31
what is associated with poor prognosis in Neuroblastoma?
N-MyC amplification (1p deletion)
32
what are the paraneoplastic syndromes associated with neuroblastoma?
(1) opsoclonus-myoclonus ataxia (multi-directional eye movements, ataxia. differential disgnosis hepatoblastoma); (2) watery diarrhoea (VIP secretion); (3) hypertension
33
What is characteristic of neuroblastoma metastatic skin lesions?
rubbing them induces central blanching and surrounding halo of erythema (lasts 30-60 min)
34
what are the stages of neuroblastoma?
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
35
what is good prognosis for neuroblastoma?
<1year old, tumour not crossing midline, hyper diploid
36
hodgkin lymphoma - age, sex, presentation
teens, females, prolonged presentation with lymph nodes, mediatinal mass, B symptoms
37
Hodgkin lymphoma - cells?
Reed Sternburg cells
38
NHL types
2 B - Burkitt & Diffuse large B cell; | 2 T - lymphoblastic lymphoma, anaplastic large cell lymphoma
39
Peak age for ALL
2-5 years of age
40
Good prognostic indicators ALL
``` 1-10years old, WCC 50 chromosomes), Trisomy 4, 10, 17, Hyperdiploid TEL AML, Translocation 12:21 (ETV-RUNX) no extramedullary disease ```
41
Bad prognostic indicators ALL
``` 10 years old, WCC >50, hypodiploid (<44 Chr), Philadelphia Chr 9:22 BRC-ABL, MLL rearrangement, extra medullary disease ```
42
Sanctuary sites for ALL
Testes and brain = worse prognosis
43
What has biggest effect of ALL prognosis
Minimal residual disease at the end of induction (>0.01% = bad)
44
T Cell ALL Philadelphia chromosome
BRC ABL1 (9,22)
45
Treatment Phladelphia chromosome
Tyrosine kinase inhibitiion (IMATINIB) - works by preventing addition of phosphate and switching “off” tyrosine kinase enzyme leading to apoptosis.
46
what other neural crest disorders are associated with neuroblastoma?
Hirschsprungs | Congenital hypoventilation syndrome
47
How does a Wilms tumour present?
young child that is WELL with abdominal mass
48
what are the risk factors for wilms tumour
``` beckwith weidemann, hemihypertrophy, urogenital malformations, NF-1, WT-1 (DenysDrash, WAGR) ```
49
what are the favourable factors for rhabdomyosarcoma
age 1-10, <5cm size, not in bladder/prostate (that is bad)
50
what is syndromes are associated with hepatoblastoma
``` beckwith weidemann, hemihypertrophy, FAP, Low Birth Weight Fetal alcohol syndrome ```
51
Which lobe is commonly affected in hepatoblastoma
right lobe
52
what might be raised in hepatoblastoma
AFP, bHCG
53
what syndromes are associated with germ cell tumours
``` gonadal dysgenesis, Klinefelters, Turners, saccral agenesis, Downs and russel silver ```
54
with testicular germ cell tumours what is the effect on fertility
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
which tumour is seen most commonly secondary to radiation
angiosarcoma
56
what tumour are plexiform fibromas in NF-1 linked to ?
malignant peripheral nerve sheath tumours.
57
what are the features in osteosarcomas
older children, local pain and swelling, metaphysis of long bones (distal femur and proximal tibia, proximal humerus), sunburst pattern on X-ray
58
what are the features of ewings
younger children, systemic symptoms, diaphysis of long bones and flat bones, translocation 11;22 in 85%
59
what are a feature of an osteoid Osteoma
pain is relieved by aspirin
60
what does a osteochondroma look like
cauliflower/stalk like appearance found near joints
61
what are the pathological findings specific for langerhan histiocytosis
birbeck granules, CD1a and CD207
62
how does multifocal langerhan histiocytosis present
mutliple bony lesions, DI, exopthalmos
63
what are the risk organs for langerhan histiocytosis
liver, spleen, bone marrow and lungs
64
what defect is linked to x-linked lymphoproliferative disease
SAP defect
65
what is the best treatment for lymph proliferative disease
Rituximab (antiCD20) and bone marrow transplant
66
what are the diagnostic criteria for HLH
``` 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
which AML is best treated with retinoic acid
APML (M3)
68
what leukaemia are Down syndrome children at risk of
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
what medication is used in CML
Imatinib - inhibits BCR-ABL tyrosine kinase
70
what is the order for best source of haemopoetic stem cell transplant
cord blood> bone marrow > peripheral blood
71
what is the order for best donor for haemopoetic stem cell transplant
HLA sibling > HLA unrelated> Relative NON-HLA matched
72
if an A patient receives donor graft from an O patient what should you remove
plasma
73
what should you remove if the patient is O and the donor is A
red blood cells
74
who are the best donors and why
males as they have no anti-Y antibodies
75
what cancer does SVC obstruction more likely to occur
T cell ALL or lymphoma
76
what is tumour lysis syndrome the highest risk
12-72 hrs post starting chemo but risk remains to 7 days
77
how does allopurinol work
inhibits xanthine oxidase (can get build up of xanthine which precipitates at pH >7.5)
78
how does rasburicase work
recombinant urate oxidase changes uric acid to allantoin
79
what must you never give with rasburicase
sodium bicarb
80
what are the indications for dialysis in tumour lysis syndrome
phosphate >10, potassium >6, volume overload, low calcium with symptoms
81
what is infection are you particularly at risk of with prolonged neutropenia
fungal infection >5-7days
82
what antibiotics should you give for febrile neutropenia
amikacin / gent or Tazocin
83
which cancer has the highest rate of secondary malignancies
hodgkins lymphoma 7%
84
what si the risk of secondary malignancy with ALL and what cancers are seen?
2-3%, | brain tumour and AML seen
85
CYCLOPHOSPHAMIDE
Alkylating agent haemorrhagic cystitis bladder cancer infertility
86
VINCRISTINE
``` acts on microtubules, M phase peripheral neuropathy jaw pain constipation burn on extravasation doesn't cause myelosuppression ```
87
ASPARGINASE
G1 phase | thrombus and DIC
88
DOXORUBICIN
anthracycline, S phase cardiac effects red urine
89
ETOPOSIDE
M phase secondary malignancy anaphylaxis myelosuppression
90
CISPLATIN
``` all phases, platin TOXIC myelosuppression hearing loss low Mg, K hair loss delayed emesis ```
91
CYCLOSPORIN
``` Calcineurin inhibitor gingival hyperplasia renal toxicity high BP high Lipids Hirsuitism ```
92
THIGUAMINE
S phase DNA analogue | Veno-occlusive disease
93
CYTOSINE ARABINOSIDE
cerebellar toxicity - ataxia, intention tremour
94
IMATINIB
QT prolongation
95
CYTARABINE
S phase cytokine storm strep infection myelosuppression

Oncology (1 decks)

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