Neuroblastoma

Question 1

What is a neuroblastoma and why do they only present in a developing embryo?

Answer 1

1. Tumour originating from the neural crest part of the sympathetic nervous system.
2. Due two abnormal proliferation of tissue.

Question 2

Which is the third most common cancer in childhood?

Answer 2

Neuroblastoma - 40% of cases in the first year of life, very rare in children over 5 years old.

Question 3

Where do half of neuroblastoma occur and where of the other common sites?

Answer 3

1. Adrenal gland
2. Abdomen and chest
3. 50% have metastasised at diagnosis

Question 4

What are the abdominal clinical features of a neuroblastoma?

Answer 4

Abdominal distension, constipation, abdominal pain.

Question 5

What are the thorax clinical features of a neuroblastoma?

Answer 5

Breathlessness, Horner’s Syndrome, SVC obstruction.

Question 6

What are the spinal cord clinical features of a neuroblastoma?

Answer 6

Leg weakness and loss of ability to walk or crawl.

Question 7

What are the endocrine clinical features of a neuroblastoma?

Answer 7

Hypertension (form catecholamines) and diarrhoea

Question 8

How is a neuroblastoma diagnosed?

Answer 8

1. USS 1st line
2. Tumour biopsy
3. Increased urinary metanephrins (HVA and VMA)
4. CT scan to assess for metastases

Question 9

What is the management and prognosis of a neuroblastoma?

Answer 9

1. Surgical excision, chemotherapy

2. 70% 5-year survival