Malignant Disease

Question 1

Factors contributing to poor prognosis in ALL

Answer 1

Age: <1y, >10y
WCC: >50
Genetic/molecular: MLL rearrangement, t(4;11), hypodiploidy
Response to initial chemo: leukaemic blasts persist in BM
MRD assessment: high

Question 2

ALL new Dx No CNS

Answer 2

Induction chemo 
Dexrazone (prev. cardiotox from doxoribicin)
Rituximab if CD20+
Trk Inhibs (imatinib for t9;22)
Additional:
- fluid therapy + allopurinol
- proph. Abx
- norethistrone to suppress periods
Correct anaemia using trx if necessary

Question 3

Induction chemotherapy in ALL

Answer 3

pred
vincristine
anthracyclines and/or L-asparginase

Question 4

Remission in ALL

Answer 4

leukaemic blasts eradicated and normal BM function

Question 5

ALL w/CNS involvement

Answer 5

Cytotoxics penetrate CNS poorly
Intrathecal chemotherappy req. to prevent CNS
Continuing chemotherapy of moderate intensity usually continued for up to 3y
High dose chemo +/- total body irradiation followed by BMT is alternative to chemo after a relapse

Question 6

Brain tumours in children

Answer 6

surgery (prevent hydrocephalus, tissue diagnosis, maximum resection)
radio/chemo

Question 7

Lymphoma in children

Answer 7

chemo +/- radio
PET monitors Rx
80% cure rate

Question 8

Neuroblastoma in children

Answer 8

most common extra-cranial tumour in children
localised primaries w/o mets can be cured by surgery alone
In some infants neuroblastoma can resolve spontaenously
Metastatic dx in older children Mx w/chemo
High dose therapy w/autologous sc rescue, surgery and radio
High risk of relapse
cure rate for metastatic is 40%

Question 9

Wilm’s tumour

Answer 9

Surgery and chemo
subsequent managament dependent on histology
5% bilat.
>80% are cured

Question 10

Retinoblastoma`

Answer 10

average age 18m
Aim to cure but preserve vision
chemo to shrink
laser treatment of retina
radio if advanced
90% cured 
many visually impaired, some req enucleation
significant risk of second malig. (sarcoma) in survivors of hereditary RB

Question 11

Soft tissue sarcoma

Answer 11

chemo, radio, surgery

65% cure

Question 12

Bone tumours

Answer 12

chemo, radio, surgery

65% cure

Question 13

Germ cell tumour

Answer 13

highly chemo sens

good prognosis