Malignant Disease

Question 1

What are the most common childhood cancers?

Answer 1

Leukaemia

Brain and Spinal

Lymphomas

Neuroblastoma

Soft tissue sarcomas

Wilms

Bone

Retinoblastoma

Question 2

What is the most common type of leukaemia in children? Who does it normally affect?

Answer 2

Acute Lymphoblastic Leukaemia - 80% of childhood leukaemias

2-5 years of age

boys > girls

Question 3

How does ALL present?

Answer 3

anaemia: lethargy and pallor
neutropaenia: frequent or severe infections
thrombocytopenia: easy bruising, petechiae

bone pain (secondary to bone marrow infiltration)
splenomegaly
hepatomegaly
testicular swelling

Question 4

What are the types of ALL?

Answer 4

common ALL (75%), CD10 present, pre-B phenotype
T-cell ALL (20%)
B-cell ALL (5%)

Question 5

What are poor prognostic indicators for ALL?

Answer 5

age < 2 years or > 10 years

WBC > 20 * 109/l at diagnosis

T or B cell surface markers

non-Caucasian

male sex

Question 6

What is contrasting to adults in children brain tumours?

Answer 6

always primary in children

60% intratentorial

Question 7

What are the types of brain tumours?

Answer 7

Astrocytoma (40%)

Medulla blastoma (20%)

Ependymoma (8%)

Brainstem Glioma (6%)

Question 8

How do brain tumours normally present?

Answer 8

often related to evidence of increased ICP

also back pain, peripheral weakness of arms or legs, bladder/bowel dysfunction

Question 9

How would you investigate brain tumours?

Answer 9

MRI

Question 10

What is a Neuroblastoma?

Answer 10

Arise from Neural Crest tissue in the adrenal medulla and sympathetic nervous system

*has tendency to spontaneously regress

Question 11

How does Neuroblastoma present?

Answer 11

abdominal mass
pallor, weight loss
bone pain, limp
hepatomegaly
paraplegia
proptosis

Question 12

What investigations would you run on suspicion of neuroblastoma?

Answer 12

urinary catecholamine levels are increased

raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
calcification may be seen on abdominal x-ray
biopsy

Question 13

What is Wilm’s tumour?

Answer 13

Embryonal renal tissue

80% present before 5 years old

Question 14

How does Wilm’s tumour present?

Answer 14

abdominal mass (most common presenting feature)
painless haematuria
flank pain
other features: anorexia, fever
unilateral in 95% of cases
metastases are found in 20% of patients (most commonly lung)

Question 15

What is Wilm’s tumour associated with?

Answer 15

Beckwith-Wiedemann syndrome
as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation
hemihypertrophy
around one-third of cases are associated with a loss-of-function mutation in the WT1 gene on chromosome 11

Question 16

What are common bone tumours?

Answer 16

malignant tumours are rare

Osteogenic sarcoma is more common than Ewing

Ewing seen more often in young children

Male predominance

Question 17

What investigations would you run for bone tumours?

What would you seen?

Answer 17

X-ray > MRI > bone scan

destruction and variable periosteal new bone formation

in Ewing - substantial soft tissue mass

Question 18

Where does Wilm’s tumour usually metastasize to?

Answer 18

Lung quite early

Question 19

What is a known cause of retinoblastoma?

Answer 19

caused by a loss of function of the retinoblastoma tumour suppressor gene on chromosome 13

around 10% of cases are hereditary - AD with incomplete penetrance

Question 20

What would you see on presentation of retinoblastoma?

Answer 20

absence of red-reflex, repalced by a white pupil (leukocoria) - the most common presenting symptom

strabismus

visual problems

Question 21

How would you manage retinoblastoma?

Answer 21

enucleation is not the only option
depending on how advanced the tumour is other options include external beam radiation therapy, chemotherapy and photocoagulation

Question 22

What are the common types of liver cancer?

Answer 22

Hepatoblastoma (65%)

Hepatocellular carcinoma (25%)

Question 23

How would liver tumours present?

Answer 23

Abdominal distension or mass

pain and jaundice

increased serum alpha-fetoprotein