malignant disease

Question 1

what are the drug specific S/E of doxorubicin, cisplatin, cyclophosphamide, vincristine

Answer 1

doxorubicin - cardiotoxicity
cisplatin - deafness and renal failure
cyclophosphamide - haemorrhagic cystitis
vincristine - neuropathy

Question 2

in leukaemia, what is a mediastinal mass characteristic of

Answer 2

T cell disease

Question 3

what are high-risk prognostic features for ALL

Answer 3

age <1 or >10
tumour load (WBC count) >50x10^9/L
cytogenetic molecular abnormalities
speed of response to initial tx - persistence of blasts in bone marrow
high minimal residual disease on assessment

Question 4

mx ALL

Answer 4

fluids
Abx
allopurinol to prevent tumour lysis syndrome
transfusions

Newly diagnosed: no cns disease
induction chemo (vincristine, pred, doxorubicin)
rituximab if CD20+ ALL
tyrosine kinase inhibitor if philadelphia Chr positive

CNS involvement:
standard chemo + intensified intrathecal chemo (to penetrate CNS)

2nd phase:
consolidation+ maintenance after remission

Question 5

What is a common complicatin of medulloblastoma

Answer 5

originates in midline posterior fossa

spinal mets as it travels through CSF

Question 6

most common paediatric brain tumour

Answer 6

astrocytoma

Question 7

how do spinal tumours present

Answer 7

back pain
incontinence
weakness of legs/arms

Question 8

Mx Hodgkin’s

Answer 8

combination chemo ABVD (adriamycin (doxo), bleomycin, vinblastine and dacarbazine)

PET scan to monitor process

Question 9

How can hodgkin and NHL present

Answer 9

painless cervical lymphadenopathy

all manner of obstructions - airway, SVC, intestinal

Question 10

what is burkitt’s

Answer 10

B-cell NHL

Question 11

what’s burkitt’s associated with

Answer 11

EBV infection

Question 12

where do neurblastomas originate from

Answer 12

neural crest tissue in adrenal medulla

sympathetic nervous system

Question 13

how does neurobalstoma present

Answer 13

pallor, weight loss
abdo mass 
hepatosplenomegaly
bone pain 
can cause SCC symptoms 
paraplegia
limp

Question 14

Ix for neuroblastoma

Answer 14

Urinary catecholamines raised
biopsy and bone marrow aspirate
MIBG - taken up by neuroblastomas so can scan it

Question 15

mx neuroblastoma

Answer 15

low risk disease (local primaries w/o metastatic spread)
observation every 3-6 weeks then decide on surgery
do chemo if refractory post surg

intermediate risk disease
chemo and surgery
(radio doesn’t work)

high risk (aggressive metastatic)
radio
surgery
BM transplant
immunotherapy

Question 16

why is rhabdomyosarcoma presentation varied

Answer 16

found in a lot of sites as it originates from primary mesenchyme

Question 17

2 paediatric bone tumours

Answer 17

osteosarcoma, ewing’s sarcoma

Question 18

x-ray signs of bone tumours

Answer 18

bone destruction and periosteal new bone formation

Question 19

mx for retinoblastoma

Answer 19

chemo to shrink then surgery

Question 20

where is gene for retinoblastoma

Answer 20

Chr 13, autosomal dominant, complete penetrance

bilateral always hereditary

Question 21

where to germ cell tumours come from

Answer 21

come from primitive germ cells which migrate from yolk sac endoderm to form gonads in embryo

Question 22

serum markers for germ cell tumours

Answer 22

alpha foetoprotein

b hCG

Question 23

what is langerhans cell histiocytosis

Answer 23

abnromal proliferation of langherans cells (antigen presenting dendritic cells)