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Tom - Oncology > General Oncology > Flashcards

General Oncology Flashcards

1
Q

What is the mechanism of action of allopurinol?

A

Inhibits xanthine oxidase
Decreased conversion of xanthine to uric acid
Prevents further creation of uric acid, does not actively reduce levels

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2
Q

What is the mechanism of action of rasburicase?

A

Converts uric acid to water soluble allantoin
Actively reduces levels

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3
Q

What are the indications for the addition of vancomycin in a case of febrile neutropenia?

A
  1. Severe sepsis
  2. Suspected resistant gram positive infection (cellulitis, obviously infected vascular devices, proven gram positive bacteraemia, known MRSA colonisation and extensive skin breaks)
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4
Q

What are the indications for addition of amikacin to a patient with febrile neutropenia?

A
  1. Systemic compromise
  2. High risk treatment protocol (AML, ALL induction or delayed intensification, infant ALL, lymphoma induction, allogeneic transplant -14 to +356) autologous transplant (day - 7 to day 30+)
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5
Q

What is the mechanism of action of Blinatumomab?

A

‘BiTE molecule- bi-specific T cell engager
One arm of antibody binds CD19, one arm binds CD3 on T cells
Induces lysis of CD19 expressing leukaemia cells
Used in precursor B acute lymphoblastic leukaemia

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6
Q

The presence of Roselthal fibre suggests which CNS malignancy?

A

Pilocytic astrocytoma

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7
Q

What is the most common location for an ependymoma?

A

Posterior fossa

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8
Q

Which of the following molecular markers is associated with a favourable prognosis in medulloblastoma?

a. WNT1
b. MYC+
c. p53
d. SHH

A

WNT1 = favourable prognosis, often arise from brainstem, very vascular
MYC+ and p53 = poor
SHH - intermediate, arise hemispheric cerebellum

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9
Q

Which pituitary hormone is most frequently impaired in craniopharyngioma?

a. TSH
b. GH
c. GnRH
d. ACHT

A

Frequency of endocrine abnormalities (GH> GnRH >TSH> ACTH)
GH = 75%, GnRH = 40%, TSH = 25%, ACTH = 25%

Often present with growth failure
Tumour arising from remnants of Rathke’s pouch

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10
Q

A child presents with headache, vomiting and visual impairment

An MRI showing calcifications in the suprasellar region.

Which tumour is most likely?

a, ATRT
b. medulloblastoma
c. craniopharyngioma
d. metastasis
e. pituitary adenoma

A

Craniopharyngioma is correct

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11
Q

A 2 year old child presents with 1 month of hypertension and fatigue
On examination they have a right sided abdominal mass that does not cross the midline
CT of the abdomen shows a renal mass with ‘claw sign’
What is the most likely cause of this presentation?

a. Wilm’s
b. Neuroblastoma

A

Answer: Wilm’s
Most common renal tumour of childhood
Less likely to cross the midline than neuroblastoma
Claw sign = sharp angles either side of the mass formed by normal parenchyma, normal parenchyma extends some way around the mass

https://radiopaedia.org/articles/claw-sign-mass

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12
Q

What are the features of WAGR syndrome?

A

Wilm’s tumour
Aniridia
Genitourinary abnormalities
Mental retardation

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13
Q

A 9 year old male presents with a swelling over their calf muscle initially thought to be a haematoma from football. After the mass did not resolve for >1 month, imaging and biopsy confirmed a rhabdomyosarcoma
Which sub type of rhabdomyosarcoma is this more likely to be?

A

A: alveolar

Embryonal subtype: younger age, more central location, mets to lungs, 40-90% EFS
70-75% of cases
Has 2 variants
Botryoid type - typically vaginal or nasaopharyngeal type
Spindle cell - paratesticular

Alveolar subtype

Usually trunk or extremities
Has one of 2 translocations:

T(2;13) pr PAX3-FKHR (FOXO1)
T(1:13) or PAX-FKHR

Older kids
More invasive
0-60% EFS

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14
Q

A 14 year old female presents with 1.5 months of right upper arm pain that sometimes wakes her from sleep and is not relieved by simple analgesia.
LMO orders plain films which shows the following:

What is the most likely diagnosis?

A

Ewing’s sarcoma
This is a mid diaphyseal lesion and has a lamellated periosteal reaction (‘onion skinning’) - these features suggest Ewing’s sarcoma
This contrasts with osteosarcoma - more likely to be sclerotic (rather than lytic), metaphyseal, located in feumur, tibia and humerus
See x -ray moth eaten, permeative lesion with Codman’s triangle on the superior part (may also have ‘sunburst’)

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15
Q

Which of the following malignancies is NOT associated with RB1 gene?

a. melanoma
b. soft tissue sarcoma
c. AML
d. osteosarcoma

A

c (to my knowledge)

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16
Q

A tall male with small testicles, limited muscular development and shy disposition presents with a mediastinal mass
What will the mass be and what is the underlying syndrome

A

Klinefelter
Predisposition for mediastinal germ cell tumours

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17
Q

Which if the following syndromes is NOT associated with hepatoblastoma?

a. FAP (familial adenomatous polyposis)
b. Li Fraumeni
c. Beckwith-Weidman
d. Klinefelter
e. Goldenhaar

A

d

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18
Q

A 2 year old boy presents with multiple painful, lytic skull lesions
Pathologically, Birbeck granules are seen, along with expression of CD207 and CD1a
What is the diagnosis?

A

Answer: LCH

19
Q

A 2 year old female with newly diagnosis ALL (acute lymphoblastic leukaemia) has the following features:

Age: 2 years 4 months
WCC at diagnosis: 17
% blasts: 21
Cytogenetics:
Hypodiploid chromosome
iAMPP21 amplication
CNS1a
End of induction MRD of 1%

Would they be:

a. low risk
b. standard risk
c. High risk

A

A: high risk

Low risk = high risk features not present, favourable cytogenetics
Favourable cytogenetics:

Hyperdiploidy (>50)
Trisomies 4, 10
ETV-RUNX protein (t12:21, previously known as TEL-AML protein)

Standard risk = high risk features not present
No favourable cytogenetics

High risk/very high risk

Age <1 or >10 years

Presentation: WCC >50 at diagnosis, CNS involvement, testicular involvement

Cytogenetic/molecular features:

Hypodiploidy (<44)
KMT2a/MLL genetic rearrangements - translocations of 11q23 (eg: t4;11)
iAMP21 amplification
Philadelphia chromosome t(9:22) BCR-ABL fusion protein - very poor prognosis
Abnormal loss of 17p, 13p
Response = failure to achieve remission by end of induction (d29) = MDR <0.01%

20
Q

Which of the following leukaemia types is known to respond to trans-retinoic acid?

a. acute pro-myelocytic leukaemia
b. pro-B acute lymphoblastic leukaemia
c. acute myelocytic anaemia
d. T cell acute lymphoblastic leukaemia

A

APML
Includes ATRA, tretinoin

21
Q

True or false?
In a 5 year old with Down Syndrome, the ratio of AML to ALL is the same as the general population

A

This is true
Ratio of AML:ALL is only inverted for the first 3 years of life

22
Q

Which of the following is NOT used as therapy in childhood CML (chronic myelogenous leukaemia)

a. Imatinib
b. hydroxyurea
c. Allogenic stem cell transplant
d. azathioprine

A

d is correct

Imatinib = tyrosine kinase inhibitor
Hydroxyurea = used whilst awaiting response to TK inhibitor in chronic phase if disabling or life threatening features present
HSCT - 80% cure rate
AZA - not for CML

23
Q

Which type of leukaemia are children with Noonan syndrome predisposed towards?

A

Juvenile myelomonocytic leukaemia (JMML)
Most patients have mutations in the RAS/MAP kinase pathway - oncogene mutations include NF1, PTPN11, KRAS, NRAS
Associated conditions include NF1 and Noonan syndrome
Half of the Noonan patients have a mutation in PTPN11
Others have NRAS, KRAS
FBE characterised by monocytosis and elevated foetal Hb
HSCT best chance of cure

24
Q

Patients with infant leukaemia are most likely to have WHICH chromosomal rearrangement?

A

MLL gene
11q23 band translocation, majority t(14;11)
Present in 80% of infant leukaemia cases

25
Q

what type of tumour is suggested by Rosenthal fibres

A

pilocytic astrocytoma

26
Q

are ependymomas primarily a) posterior fossa or b) supratentorial?

A

posterior fossa 70%

27
Q

which of the following is associated with the BEST prognosis in medulloblastoma?

a. group 3
b. group 4
c. SHH
d. WNT

A

WNT best prognosis
Group 3 worst

28
Q

what phase of the cell cycle is associated with chromatides being pulled apart by centromeres?

A

Anaphase

29
Q

How many chromatids are present during a cell in metaphase

A

96 chromatids, 46 chromosomes, 23 pairs of chromosomes

30
Q

In what syndrome is there a defect in the p53 tumour suppressor gene?

A

Li-Fraumeni syndrome
osteosarcoma, soft tissue sarcoma, acute leukaemia, breast and brain cancer, adrenal cortical tumours
50%
Individuals with LFS have an approximately 50% of developing cancer by age 40, and up to a 90% percent chance by age 60, while females have nearly a 100% risk of developing cancer in their lifetime due to their markedly increased risk of breast cancer

31
Q

This child has short stature and their rash appears to get worse with sunlight. They are receiving treatment for leukaemia.
What is their underlying diagnosis?

A

Bloom syndrome

https://dermnetnz.org/topics/bloom-syndrome#:~:text=Bloom%20syndrome%20is%20an%20autosomal,manifest%20symptoms%20of%20the%20disease.

32
Q

aniridia and abdominal mass, what is the diagnosis

A

WAGR
Wilm’s, aniridia, genitourinary abnormalities, renal impairment

33
Q

a patient have a bony sarcoma. It is radiation sensitive. It is more likely to be

a. Ewing’s
b. osteosarcoma

A

A = Ewing’s more radiation sensitive
This is what helped James Ewing differentiate this sarcoma from osteosarcoma in 1972

34
Q

A bony tumour is analysed with FISH and is found to have translocation via break apart probe at 22q12-13
What tumour is this likely to be?

A

Ewing’s
EWSR1 gene located at 22q12.2
Classic (but not exclusive) translocation is t11;22)

35
Q

what tumour is associated with loss of INI1?

A

Atypical rhabdoid teratoma

36
Q

what tumour is associated with t(1;13) or t(2;13) = PAX-FKHR

A

alveolar rhabdomyosarcoma

37
Q

What malignancies are APC mutations associated with?

A

Adenocarcinoma of the colon
Hepatoblastoma

38
Q

which form of germ cell tumour is most likely to have an elevated B-HCG?

A

Choriocarcinoma

39
Q

which form of germ cell tumour is most likely to have an elevated AFP?

A

yolk sac tumour (endodermal sinus tumour)

40
Q

which type of chemotherapy is known to be highly active against germ cell tumours?

A

Platinum (carboplatin and cisplat)

41
Q

what does the antibody treatment dinutuximab target?

A

Neuroblastomas express an antigen called disialoganglioside 2 (GD2)
Also present on nerve cells

42
Q

Patient with AML
Recent cytarabine
Presents with mucositis, septic

?most likely organism

staph epi
staph aureus
herpes simplex
strept viridans

A

strept viridans

https://www.slaop.org/pdf/569Infecao%20High%20frequency%20of%20streptococcal%20bacteraemia%20during%20childhood%20AML%20therapy%20irrespective%20of%20dose%20of%20cytarabine.pdf

43
Q

what tumour is most likely to be associated with congenital central hypoventilation syndrome?

A

Neuroblastoma

44
Q

the Philadelphia chromosome is a translocation between which two chromosomes?

A

t(9:22)
BCR-ABL

Tom - Oncology (1 decks)

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