Oncology

Question 1

What part of the cell cycle do anti-metabolites act on and name 3 examples of these drugs

Answer 1

Inhibit S phase = DNA cycle. Examples are methotrexate, 6-mercaptopurine, thioguanine, Ara-C (Cyarabine), 5-flourocil, Hydroxyurea

Question 2

What are the side effects of anti-metabolites?

Answer 2

Myelosuppression, mucositis, dermatitis. Ara-C can cause cerebellar toxicity. ** Think Methotrexate**

Question 3

Why is it important to measure TPMT levels?

Answer 3

TPMT is used to metabolise Azathioprine/6-mercaptopurine. If there is a TPMT inactivating mutation, can cause severe toxicity with standard doses as dose builds up.

Question 4

Where does Bleomycin act?

Answer 4

G2 phase

Question 5

What is the action of Actinomycin?

Answer 5

Inhibits transcription

Question 6

What do anti-DNA antibiotics do? What is their mechanism of action?

Answer 6

Bind to DNA, intercalation, cleave DNA strands, inhibit DNA transcription
End in -cin. Ie Bleomycin, Actinomycin, Doxorubicin

Question 7

Where do topoisomerase inhibitors act?

Answer 7

Cause DNA strand breaks in late S/early G2 phase

TopoisomeraSSe- S phase

Question 8

What are the two types of topoisomerase inhibitors?

Answer 8

Non-intercalating ie Etoposide, Irinotecan
Intercalating: Anthracycline ie Doxorubicin and Daunorubicin

Question 9

What is the mechanism of action specifically for doxo and daunorubicin?

Answer 9

Topoisomerase inhibitor, intercalating agent and reactive oxygen species
(triple threat)

Question 10

What are the side effects of doxo and daunorubicin

Answer 10

RUBY- RED HEART
Red urine, tissue necrosis, arrythmias, DILATED cardiomyopathy

Question 11

How and where in the cell cycle does Vincristine work?

Answer 11

Binds to Tubulin and inhibits M phase
VIMCRISTINE

Question 12

What are the side effects of Vincristine

Answer 12

neurotoxicity (peripheral, cranial, autonomic), SIADH, constipation/ileus, jaw pain

FATAL IF GIVEN INTRATHECAL

Question 13

What are the side effects of aspariginase?

Answer 13

Coagulopathy (stroke, sinus thrombosis), pancreatitis, diabetes mellitis, allergy/anaphylaxis

Question 14

How do alkylating agents work and what are some examples?

Answer 14

Act by bonding alkyl group to DNA which induces apoptosis
Cyclophosphamide

Question 15

Side effect of Ifosfomide/Cyclophosphamide?

Answer 15

Haemorrhagic cystitis, infertility, nephropathy
IFOSPHAMIDE can cause encephalopathy due to metabolites which cross the BBB. Treat with methylene blue.

Question 16

Side effect of Cis/Carboplatin?

Answer 16

Severe delayed emesis, nephro and ototoxicity,

Question 17

Side effect of busulfan?

Answer 17

Lowered seizure threshold, veno-occlusive disease

Busulfan BLOCKS vessels

Question 18

Side effect of Etoposide?

Answer 18

HIGHEST risk for secondary malignancy and second highest for allergy.

Question 19

Which chemotherapy agents are the worst for fertility?

Answer 19

Alkylating agents et ifosfamide, busulfan, cyclophosphamide

Question 20

What ions/minerals are involved in tumour lysis syndrome?

Answer 20

Ca, Phos, K, Uric acid. HIGH uric acid, potassium and phosphate. LOW calcium. (precipitates with phosphate). LDH also monitored for degree of cell lysis.

Question 21

What is the mechanism of action for allopurinol vs rasburicase for treatment of TLS?

Answer 21

Allopurinol blocks xanthine oxidase inhibitor so reduced urate formation. Rasburicase catabolises uric acid into allantoin to make it more easily excretable in kidneys.

Question 22

What cancers are commonly associated with superior vena cava obstruction?

Answer 22

T-cell ALL, lymphoma, germ cell tumours (teratoma)

Question 23

What cells/organs synthesise AFP?

Answer 23

Fetal yolk sac, liver and intestines. So can be elevated in acute liver disease, biliary atresia, ataxic telangectasia, tyrosinaemia, epithelial liver tumours (hepatoblastoma)

Question 24

What is the proportion of B:T cell in ALL?

Answer 24

Pre-B is 70%, T-cell is 30%

Question 25

What are the high risk factors in ALL?

Answer 25

1. Age <1 or >10 2. WCC > 50 3. Congenital mutations ie BCR:ABL, 4:11, hyPOploidy 4. CNS diseases (blasts on LP)- essentially any extramedullary disease 5. Response to induction

Question 26

What do AMLs present with?

Answer 26

Chloroomas (leukocytic deposits), hyperleukocytosis

Question 27

What are the risk factors for AML

Answer 27

Cytogenetics and response to treatment

Question 28

What is the mutation (and location) for acute promyelocytic leukemia?

Answer 28

PML-RARA mutation on 15;17

Question 29

What is the treatment for APML?

Answer 29

Arsenic/Vit A derivative. Commonly will have cytokine storm on starting treatment (like SIRS response)

Question 30

What syndrome is associated with JMML? What is the mutation?

Answer 30

Noonan's commonly associated, mutation is PTPN11 NF1 also associated

Question 31

What is the classic presentation for HODGKIN's lymphoma?

Answer 31

B symptoms and neck/mediastinal disease. BIMODAL

Question 32

What staging is used for Hodgkin's lymphoma?

Answer 32

Ann-Arbour staging

Question 33

Which cancer has Reed Sternberg cells?

Answer 33

Hodgkins

Question 34

What is the distribution for NHL? (ignore this questoin, error)

Answer 34

Bimodal

Question 35

What are the common NHL?

Answer 35

Burkitts and diffuse large B cell lymphoma- DLBCL

Question 36

What is a known trigger for Burkitts

Answer 36

EBV

Question 37

What is the treatment for NHL?

Answer 37

RItuximab (anti-CD20)- note high tumour lysis syndrome risk

Question 38

What do patients with T-cell lymphoblastime leukemia OR pre-T cell lymphoma present with?

Answer 38

Teenagers with SOB, BM failure and mediastinal mass on CXR due to thymus involvement

Question 39

Staging for NHL?

Answer 39

St Judes

Question 40

What is the mutation in retinoblastoma? And presentation?

Answer 40

RB1, white reflex with reduced VA and strabismus

Question 41

What embryonic tissue is nephroblastoma (Wilms) derived from?

Answer 41

MESOnephros (NephroblastoMA = MEsonephros)

Question 42

What the the gene associated with Wilms tumour?

Answer 42

WT1

Question 43

What are the syndromes associated with Wilms

Answer 43

Overgrowth so 1. Beckwith Weideman. 2. WAGR (DELETION OF WT1) and Denys Drash (Truncation of WT1) 3. Fanconi anaemia 4. Pearlman syndrome 'WAGR deleted, Denny's has a trunk'

Question 44

What is WAGR?

Answer 44

Wilms Aniridia- abnormal development of the eye Genitourinary- no testes Retardation

Question 45

What are the 3 features of Denny's Drash?

Answer 45

Gonadal dysgenesis, wilms and nephropathy

Question 46

What is the age presentation of Wilms vs neuroblastoma?

Answer 46

Neuroblastoma <2, wilms 2-4

Question 47

What are imaging findings of neuroblastoma vs nephroblastoma?

Answer 47

Claw hand in kidney and calcification in NB

Question 48

What is the most common extracranial tumour?

Answer 48

Neuroblastoma

Question 49

What cells are mutated in neuroblastoma?

Answer 49

Neural crest cells

Question 50

What are the 3 syndromes associated with neuroblastoma?

Answer 50

Pepper syndrome, horner syndrome (ptosis, myosis, anhydrosis), opsoclonus-myoclonus ataxia syndrome

Question 51

What is the relationship between NMYC gene and NB outomes?

Answer 51

HIGH levels of NMYC= NOT good for prognosis. Think N for NMYC and N for Neuroblastoma = N for not good MYC involved in EBV associated lymphoma!

Question 52

What two syndromes are Rhabdomyosarcomas associated with?

Answer 52

LiFraumeni and NF1. Tumour derived from skeletal muscle primitive cells

Question 53

What is the name of tumours with blue cell?

Answer 53

1. Neuroblastoma, 2. Ewing’s sarcoma 3. Peripheral primitive neuroectodermal tumours 5. Non-hodgkin’s lymphoma 6. Soft tissue sarcomas 7. Megakaryoblastic leukaemia

Question 54

What is the radiological sign of ewings?

Answer 54

Periosteal reaction can be peeling onion or starburst sign

Question 55

Where is the location of Ewings vs osteosarcoma?

Answer 55

Ewings in diaphysis (shaft) while osteo is at the growth plate

Question 56

What is the definition of langerhan cell histiocytosis?

Answer 56

Disorder of one or more osteolytic bone lesions with histiocyte infiltration

Question 57

What is holoproscencephaly

Answer 57

failure of prosecephalon or forebrain to develop. Presents with microcephaly, hydrocephalus, facial abnormalities and cleft lip/palate Associated with Trisomy 13 and sonic hedgehog pathway ;Hollow at the front'

Question 58

What is Miller Diekr syndrome

Answer 58

LISSENcephaly, distinctive facial features, hypotonia, seizures/neurological symptoms

Question 59

What is associated with the sonic hedgehog pathway?

Answer 59

Holoprosencephaly, basal cell carcinoma and sporadic tumours *Has been past question*

Question 60

What is the sonic hedgehog pathway?

Answer 60

Sonic Hedgehog (Shh) signalling pathway is one of the major trafficking networks that regulates the key events during developmental processes i.e growth and patterning of multicellular embryos.

Question 61

What is the origin of a cranipharyngioma?

Answer 61

Remnant of Rathkes pouch

Question 62

What are the endocrine effects of a craniopharyngioma?

Answer 62

precocious ouberty, pituitary deficiencies

Question 62

What are the endocrine effects of a craniopharyngioma?

Answer 62

precocious ouberty, pituitary deficiencies

Question 63

What is a common cause/trigger for post-transplant lymphoprolifeartive disorder?

Answer 63

EBV

Question 64

Which blue cell tumour presents in pseudorosette formation

Answer 64

Neuroblastoma

Question 65

Which two cancer meds does allopurinol interact with?

Answer 65

Methotrexate and Cyclophosphamide

Question 66

Which medication used for tumour lysis syndrome is better for kidney - allopurinol or rasburicase and why

Answer 66

Rasburicase because helps convert uric acid to soluble allantoin via urate oxidase. Allopurinol just stops further uric acid formation but can lead to deposition of uric acid in kidneys