Adrenal Disorders - nomas

Question 1

What is an adrenal adenoma?

Answer 1

A benign tumour arising from the cells of the adrenal cortex

Question 2

What are some conditions caused by functioning adrenal adenomas?

Answer 2

1. Cushing’s syndrome
   = excess cortisol
2. Conn’s syndrome
   = primary hyperaldosteronism

Question 3

What percentage of adrenal adenomas are non-functioning and asymptomatic?

Answer 3

Around 95%

Question 4

What is the typical size and appearance of an adrenal adenoma?

Answer 4

1. Solitary, small (2–3 cm)
2. Well-circumscribed
3. Encapsulated, and bright yellow due to high lipid content

Question 5

In an adrenal adenoma, what happens to ACTH levels?

Answer 5

ACTH is low due to negative feedback from autonomous cortisol production

Question 6

What is the management for a small, non-functioning adrenal adenoma?

Answer 6

It can be left alone

Question 7

What medication is used post-operatively or for metastatic disease?

Answer 7

Mitotane

= an adrenolytic drug that suppresses adrenal hormone production

Question 8

What is a neuroblastoma?

Answer 8

A malignant neuroendocrine tumour of the sympathetic nervous system originating from neural crest cells

Question 9

What is the cellular origin of neuroblastoma?

Answer 9

Neural crest cells

Question 9

At what age is neuroblastoma usually diagnosed?

Answer 9

Median age is around 18 months; about 40% are diagnosed in infancy

Question 10

What genetic markers are associated with poor prognosis in neuroblastoma?

Answer 10

N-myc amplification and telomerase expression

Question 11

You are reviewing a 4-year-old boy with constipation in primary care. When examining his abdomen you notice a small mass in his left lower abdomen. It does not seem to cause him any distress when you palpate it.

Mum tells you that a GP had previously noticed this, but reassured her that it is most likely some stool that is palpable due to constipation.

He is otherwise well and mum has no particular concerns.

Which one of the following is the most appropriate next step in management?

Answer 11

Discuss him with the on-call paediatric registrar

= For a child with a palpable abdominal mass or unexplained enlarged abdominal organ: refer very urgently (<48hr) for specialist assessment for neuroblastoma and Wilms’ tumour