Miscellaneous

Question 1

Retinoblastoma 
Cells affected 
Gene
\_\_\_ hit hypothesis
Inheritance 
Inherited form- age of onset
Acquired- onset 
Associated Ca, which type

Answer 1

Retinal nucleated cells- any layer
Rb1 on Ch13
Inactivates tumour suppressor gene
2 hit hypothesis 
Inherited: <5 mo (40% cases)
Acquired: <2y (60%)- all unilateral

Osteosarcoma, melanoma, sarcoma association with inherited form
Avoid XR

Question 2

Differential dx leukocoria

Answer 2

Retinoblastoma 
Cataract 
ROP
Large chorioretinal coloboma
Persistent hyperplastic vitreous 
Retinal dysplasia

Question 3

Rhabdomyosarcoma

Associated syndromes
Genetic RF

Answer 3

Li Fraumeni
BWS
NF
Chromosomal translocations t(2;13) common

Question 4

Rhabdomyosarcoma

Common sites

Answer 4

Head and neck (40%)
GUT (40%)
Limbs (20%)
Trunk (10%)

Question 5

Rhabdomyosarcoma

Presentation
Spread
Histology

Answer 5

Mass, pain, loss of function

Lymph nodes
Lungs
Bone
BM

Small round blue cell

Question 6

Spinal cord compression

7 cancer possible

Emergency management

If Ewings or neuroblastoma- emergency mgmt

Answer 6

Rhabdomyosarcoma 
Osteosarcoma 
Ewing 
Neuroblastoma
Hodgkin 
Sarcoma
GCT

IV dexamethasone

RTx

Question 7

BMT

Most common indication

When can immunosuppression be stopped

Answer 7

ALL -50%
AML- 20%
Immunodeficiencies 10%
Fanconi anaemia 5%

6–12 mo

Question 8

Acute GvHD
Time frame
Pathogenesis
Risk factors

Answer 8

First 8 weeks
CD8 T cells from graft attach host cells due to HLA mismatch
Engrafted host cells release TNF, IL-1
NK cell mediated cell death

Less HLA match
Increased age
Unrelated donor
Peripheral >BM >cord blood 
Rtx dose
Acute or relapsed malignancy at time of transplant

Question 9

Chronic GvHD
Timing 
Mimics \_\_\_
Skin
GIT
Conjunctiva
Mucosa
Lungs 

Limited
Extensive

Answer 9

>2 months 
Scleroderma 
Rash, moisture, elasticity, alopecia 
Cirrhosis 
Sicca
Leukoplakia 
Bronchiolitis obliterans

Skin + liver
Other organs

Question 10

Chronic GvHD

RF

Answer 10

Increased age
Significant acute gvhd
Female donor of high parity
Buffy coat transfusion

Question 11

Veno-occlusive disease
Clinical features
Prognosis- which organs fail
Rx

Answer 11

Jaundice
Tender hepatomegaly
Hypoalbuminaemia, ascites & weight gain

Liver kidney
Poor prognosis

Difibrotide

Question 12

Secondary tumours

Most common in 1st year
Most common solid tumours

RFs

Risk of relapse once 5 y post treatment without relapse

Answer 12

NHL
Thyroid + brain

Young age
Total body irradiation
ATG

Same as general population

Question 13

TLS

Release of…

Calcium…

Starts, lasts

Answer 13

Phosphate
Uric acid
Potassium

Calcium binds phosphate

Onset in 1st 24h, lasts 4-5 days

Question 14

TLS

RFs

Tumour type
Labs

Answer 14

Tumour burden
NHL
- T cell lymphoma with large mediastinal mass
- Burkitts 
ALL with HSM
Bulky abdominal tumours 

Elevated uric acid
LDH
WCC
Poor urine output