Oncology

Question 1

Histopath findings: Reed Sternberg Cells

Answer 1

Hodgkin Lymphoma

Question 2

Philadelphia Chromosome- BCR-ABL fusion (9:22)

Answer 2

B-Cell ALL- POOR prognostic sign
- Poor response to chemo is the worst prognostic sign

Question 3

Mutation- PTPN11 (associated with Noonan’s)- can be somatic or germline

Answer 3

JMML

Question 4

GATA1 mutations/ ALL or AML

Answer 4

Down’s Syndrome/T21

Question 5

Histopath findings: Starry sky appearance on film

Answer 5

Burkitt Lymphoma

Question 6

Histopath findings: Auer rod

Answer 6

AML

Question 7

Signs of VOD?

Answer 7

Jaundice and hyperbilirubinaemia
Right upper quadrant pain, generally with tender hepatomegaly.- raised transaminases
Weight gain and ascites

DDx: portal obstruction- expect splenomegaly

Question 8

DDx anterior, posterior, superior & middle mediastinal masses

Answer 8

Posterior: Neuroblastoma (adjacent to paraspinal ganglion), thoracic meningiocele, aortic aneurysm

Superior: cystic hygroma, thymic mass, teratoma, ectopic thyroid, oesophageal lesions, AVM

Anterior: epithelial tumor, lymphoma, thymoma

Middle: lymphoma, lymphadenopathy, bronchogenic/pericardial cysts

Question 8

Blood findings in TLS & management

Answer 8

High K
High Phos
High uric acid
Low calcium

1. Hyperhydration/HCO3
2. Rasburicase= promotes the conversion of uric acid to allantoin, which is less harmful and easily excreted
3. Allopurinol = xanthine oxidase inhibitor, TLS prophylaxis

Question 9

What patients need irradiated blood cells?

Answer 9

Patients at risk of GVHD:
* Transplant.
* Hodgkin lymphoma, aplastic anaemia.
* Monoclonal antibodies or therapeutic antibodies.
* Granulocyte transfusions.

Question 10

Denys-Drash & Frasier syndrome and tumor associated?

Answer 10

Wilms (WT1)

• Early-onset renal failure
• XY gonadal dysgenesis

Frasier

Question 11

M phase chemo drugs?

Answer 11

Vincristine/blastine
Docetaxel/paclitaxel

Question 12

S phase chemo drugs?

Answer 12

Antimetabolites:
- Folate analoges: methotrexate
- Purine analogues: fludarabine, mercaptopurine, thioguanine
- Pyrimidine analogues: cytarabine, 5-FU

Misc: hydroxyurea

Question 13

G2 phase chemo drugs?

Answer 13

Topoisomerase 1 inhibitors: etopaside
Bleomycin

Question 14

G1 phase chemo drugs?

Answer 14

Asparaginase/PEG

Question 15

Common pathogens in febrile neutropenia
(patients with Rx AML, cytarabine, awaiting transplant)

Answer 15

Strep mitis (CONS- occupies port/line)

Question 16

Chemotherapies at risk of 2nd malignancy

TAAAT

Answer 16

Topoisomerase inhibitors

Alkylating agents (cyclophosphamide)- biggest risk

Anthracyclines (doxorubicin)

Antimetabolites (MTx, thiopurine, mycophenylate)

(anti)Tubulin (vincristine)

Question 17

Cancer with highest likelihood of secondary malignancy

Answer 17

Hodgkin lymphoma- 18.5x increase

• AML (3yrs)
• Breast
• Thyroid
• Soft tissue
  (14yrs solid)

Question 18

Mutation and Ca predisposition in T21?

Answer 18

AML in Trisomy 21
* Secondary to GATA1 mutation
* 150x increased risk of AML
* Increased sensitivity to chemotherapy, survival >90% age <4years

Question 19

Favourable prognosis genes in AML

Answer 19

RUNX
CBFb-MYH
NPM
CEBPA

Question 20

Poor prognosis gene mutations in AML

Answer 20

Monosomy 5,7
FLT/ITD
KMT2A
NUP
DEK

Question 21

Dx on this Xray?

Answer 21

Ewings sarcoma

Question 22

Dx on this XR?

Answer 22

Osteosarcoma
5-25yrs, M>F
- Most common bone tumor
- Most commonly long bone metaphyses (distal femur/prox humerus, tibia, pelvis and skull)
- Formed by osteoblasts/mesenchymal cells

XR: dense sclerosis at metaphysis with soft tissue expansion- ‘sunburst appearence’

Question 23

Explain 2 hit hypothesis with retinoblastoma

Answer 23

1. Germline mutation of one of the RB1 tumour suppressor gene
2. Second mutation in the remaining RB1 allele of retinal cells.

Question 24

Most important factors impacting survival for donor cell transplant in 8/8 HLA matched unrelated donor?

Answer 24

1. full HLA match of the donor at HLA-A, HLA-B, HLA-C and HLA-DR loci- overall survival drops 10% with each mismatch 2. Age 5.5% increase in risk for each 10yr increment 3. CMV- seronegative = better survival 4. ABO compatability not required however can lead to complications if incompatible

Question 25

Which is more common AML/ALL? Features to suggest

Answer 25

ALL 75% AML 20% - Gum hypertrophy - Syndromic association Both have HSM, pancytopenia with blasts

Question 26

Cell markers AML vs ALL

Answer 26

AML - CD7, 3 - MPO ALL - CD34, CD 19, CD10, CD 79a, CD33