PAEDIATRIC ONCOLOGY

DR SIMON ATTARD MONTALTO

Overall incidence approx. 1 in 600

Tumours result in 2nd commonest cause of death between 1-15 years.

Tumour	% of total	Incidence
Leukaemia	32	1.0 in 2,500
Lymphoma	12 HD5 NHL7	1.0 in 10,000
CNS	20	6.5 in 10,000
Neuroblastoma	7	>1.0 in 100,000
Sarcomas	6.5	1.0 in 100,000
Wilms	6	<1.0 in 100,000
Retinoblastoma	4	0.5 in 100,000
Bone	5 OST 3 EW 2	>0.5 in 100,000
Others	7.5	>1.0 in 100,000

LEUKAEMIA

1. Lymphoblastic           up to 70% long term survival common ALL

2. Myeloblastic              up to 45% long term survival

Treatment: chemotherapy, radiotherapy

Prognosis (ALL), worse if…

§ <1 year

§ presenting WCC >50 x 109/l

§ presenting Hb not low

§ boys

§ L1 > L2 > L3 morphology

§ cALL > T > B > null cell ALL

§ cytogenetics (e.g. translocations)

WILMS' TUMOUR

80% 'cure' if stage I or II

1% familial

Associations:

aniridia

hemihypertrophy

Beckwith Wiedeman Syndrome

renal, genital abnormalities

Treatment

surgery

radiotherapy

chemotherapy

RETINOBLASTOMA

>90% are cured

aim is to preserve/salvage vision

Treatment:

surgery

radiotherapy - cryotherapy, plaques, ext. beam

chemotherapy - tumour at resection margin

orbital recurrence

distant metastases

BRAIN TUMOURS

Prognosis – variable, depends on

site

histology

spread to/outside CNS

SARCOMAS

2-6 year olds

multiple sites of origin

Treatment:

surgery

radiotherapy

chemotherapy

Prognosis

Stage I              90% 2-year survival

Stage II                        <40% 2-year survival

Histology

Site of primary (trunk, retroperitoneal worse)

OSTEOSARCOMA

15-19 years

increased risk in irradiated bones

femur > tibia > humerus

Treatment:

surgery

chemotherapy

Prognosis:

surgery alone                20% 10 year survival

surg & chemo               50% 10 year survival

with mets                      <10% 10 year survival

EWINGS TUMOUR

metastases at presentation in 30%

Treatment:

surgery

radiotherapy

chemotherapy

Prognosis:

site and size all-important

overall 5 year survival <50%

+ mets 5 year survival <20%

late relapsing tumour

NEUROBLASTOMA

50% of cases are less than 2 years

arise from neuro-ectodermal tissues

Associations:

neurofibromatosis

phaeochromocytoma

hemihypertrophy

heterochromia

Treatment:

chemotherapy

surgery

MIBG

Prognosis: most are stage IV at diagnosis, tendency to recur

if <1 year at diagnosis - 56% alive at 5 years

if >1 year at diagnosis - 20% alive at 5 years

FUTURE

1. Strive to enhance and optimize treatment regimens

2. Improve treatment to reduce morbidity and long term effects

3. Improve shared care, treatment facilities, education etc

4. Pursue research avenues

"To cure sometimes. To relieve often. To comfort always" (15th century French saying)