Oncology

Question 1

What are the 2 Types of ALL

Answer 1

b-cell
T-cell

Question 2

Whats the pathophysiology of ALL?

Answer 2

stem cell DNA mutations cause hyper proliferation of WBC - excessive production of immature blast cells

Question 3

Environmental risk factors of developing cancer in children

Answer 3

Radiation
Infection
Parental smoking during pregnancy

Question 4

Known genetic risk factors

Answer 4

Down’s syndrome- increase risk of leukaemia
Familial retinoblastoma

Question 5

Which types of childhood cancers are caused by infections

Answer 5

Berkitts lymphoma
Hodgkin’s lymphoma
Nasopharyngeal carcinoma - EBV
Hepatocellular carcinoma - hep B
Kaposi sarcaom HHV8

Question 6

What is a paraneoplastic phenomena

Answer 6

Symptoms that are not directly attributable to the tumour but arise as part of body’s response to disease

w.g. pain, lethargy, pallor

Question 7

What is opsoclonus-myoclonus -ataxia syndrome?

Answer 7

Dancing eyes
Example of paraneoplastic phenomenon
Caused by neuroblastoma

Abnormal eye jerking

Question 8

What is the treatment for opsoclonus-myoclonus-ataxia syndrome?

Answer 8

immunosuppression

Question 9

Presentation of pancytopenia in children with malignancy

Answer 9

Pallor/ lethargy - low Hb
Recurrent fever/ infection - low WBC
Bruising or petechiae - low PLTS

Question 10

Lymphadenopathy/ unexplained mass presentation

Answer 10

Lymph node with diameter greater than 2cm
Non-tener, ruberò, hard or fixed
Supraclavicular or axillary location
Systemic symptoms - fever/ weight loss/ night sweats

Question 11

Respiratory symptoms

Answer 11

Orthopneoa
Reduced a/e - pleural effusion

Question 12

Presentation if bone and joint pain

Answer 12

Persistent back pain
Night pain
Reluctance to weight-bear or new limp

Question 13

Presentation if abdominal mass

Answer 13

General malaise - neuroblastoma
HTN - Wilms or neuroblastoma

Question 14

Presentation if raised intracranial pressure

Answer 14

Heachache
Papilloedema
III and VI cn palsies

Question 15

Neurological signs

Answer 15

cerebellar signs
Visual disturbances

Question 16

What should you investigate if suspecting liver tumour or malignant germ cell tumour?

Answer 16

Alpha-fetoprotein AFP
b-human chorionic gonadotrophin B-HCG

Question 17

What is AFP associated with

Answer 17

Epithelial liver tumours
Hepatiblastoma and hepatocellular carcinoma
Yolk sac tumours
Embryonaly carcinoma

Question 18

What is G1 in the cell cycle

Answer 18

‘Gap phase’ - synthesis of the components required for DNA synthesis

Question 19

What is S phase in cell cycle

Answer 19

DNA synthesis phase before second gap phase G2

Question 20

What happens in G2

Answer 20

Cell prepares for mitosis

Question 21

What happens in M phase of cell cycle

Answer 21

Final phase - actively replication cells is mitotic phase

Question 22

What is G0 in cell cycle

Answer 22

Rest phase

Question 23

How does vincristine work?

Answer 23

block the function of the spindle apparatus required for mitosis

Question 24

How does etoposide work?

Answer 24

Causes DNA stand breaks or directly damage DNA by forming crosslinks

Question 25

Where does antimetabolites work?

Answer 25

S phase - blocks synthesis of normal nucleic acids required for DNA synthesis

Question 26

Name antimetabolites

Answer 26

Methotrexate Cytarabine Mercaptopurine

Question 27

What cancers are at increase risk of Tumour lysis syndrome

Answer 27

High-count ALL and NHL (B cell)

Question 28

Cause of tumour lysis syndrome

Answer 28

Rapid lysis of malignant cells on initiating chemotherapy

Question 29

Features of tumour lysis syndrome

Answer 29

Hyperkalaemia Hyperuricaemia Hyperphosphataemia Hypocalcmaeia

Question 30

What cancers are at risk of hyper viscosity syndrome?

Answer 30

High-count leukaemia

Question 31

Cause of hyper viscosity

Answer 31

Sludging of venous blood in small vessels Large number of circulating blast cells -> poor perfusion -> stroke and death Blood transfusion dangerous

Question 32

Raised intracranial pressures features

Answer 32

headaches vomiting ataxia papilloedema

Question 33

Febrile neutropenia features

Answer 33

temp >38 neutrophil count <1

Question 34

Stage 1 Wilm's Tumour

Answer 34

Tumour confined to kidney and is completely removed at time of surgery

Question 35

Stage 2 Wilms Tumour

Answer 35

Tumour has broken through the kidney lining but still removed by surgery

Question 36

Stage 3 will's tumour

Answer 36

Some of the tumour if left behind at time of surgery- tumour breaks or complete surgical removal sis not possible

Question 37

Stage 4 Wilm's tumour

Answer 37

Spread to other parts of body - lungs or lymph nodes

Question 38

Stage 5 Wilm's tumour

Answer 38

Tumour in both kidneys

Question 39

Which Investigation differentiates B cell from T cell NHL

Answer 39

flow cytometry - identify presence of absence of protein markers on cell surface

Question 40

What genetic syndromes are Wilm;s Tumours ass. with?

Answer 40

Beckwith-Wiedemann syndrome WAGR syndrome microdeletions of short arm in chromosome 11 (11p)

Question 41

What would results show of biopsy of ?hogkins lymphoma

Answer 41

lymphocytic and histolytic cells

Question 42

Which lineage of cells does Hodgkin's lymphoma come from?

Answer 42

clonal transformation of B- cells

Question 43

What are the four subtypes of Hodgkin's lymphoma?

Answer 43

Nodular sclerosing - most common Mixedixed cellularity Lymphocyte rich Lymphocyte depleted - rare in children

Question 44

Presentation of Hodgkin's lymphoma

Answer 44

Painless enlargement of supraclavicular lymph nodes B- symptoms Splenomegly

Question 45

Lymph nodes biopsy in Hodgkin's lymphoma

Answer 45

Reedsternberg cells

Question 46

Staging of Hodgkin's lymphoma

Answer 46

Stage 1 - single lymph node or extranodal site Stage 2 - 2 or more lymph nodes on same side of diaphragm Stage 3 - Lymph-node involvement on both sides of diaphragm Stage 4 - Disseminated or multiple involvement of extra nodal sites

Question 47

What percentage of lymphoblasts in bone marrow is needed for a definitive diagnosis of ALL

Answer 47

20% or more

Question 48

Prognosis is worse for.....

Answer 48

<1 year olds

Question 49

What Xray findings are in long bones with ALL

Answer 49

osteolytic medullary lesions

Question 50

What is a neuroblastoma?

Answer 50

Embryonal cancer of peripheral sympathetic nervous system

Question 51

What is a good prognostic indictator in neuroblastomas?

Answer 51

high levels of TRk gene give a favourable outcome

Question 52

Poor prognosis of ALL

Answer 52

Male Age <2 WBC >50 T-cell phenotype CNS disease

Question 53

Pathophysiology of hereditary spherocystosis

Answer 53

Abnormality of cytoskeleton of RBCS

Question 54

what does osmotic fragility test show

Answer 54

increase hamolysis not specific to hereditary sphereocystosis

Question 55

What would you see on blood film in patients with hereditary spherocysotis

Answer 55

spheroidal cells

Question 56

What would you see in neuroblastoma

Answer 56

VMA or hva (URINE)

Question 57

What gene is related to retinoblastoma

Answer 57

RB1 on 13 q chromosome

Question 58

What is histological signs of ALL

Answer 58

blast cells high nucleus to cytoplasm ratio

Question 59

What is burrkitt lymphoma

Answer 59

type of non-hodgkin lymphoma

Question 60

What gene is ass with bruit lymphoma

Answer 60

MYC gene