Phaeochromocytoma and adrenal lesions Flashcards

1
Q

What is a Phaeochromocytoma?

A

Neuroendocrine tumour of the chromaffin cells of the adrenal medulla. Hypertension and hyperglycaemia are often found.

  • 10% of cases are bilateral.
  • 10% occur in children.
  • 11% are malignant (higher when tumour is located outside the adrenal).
  • 10% will not be hypertensive.
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2
Q

Familial cases are usually linked to the Multiple endocrine neoplasia syndromes (considered under its own heading).

A
  • Most tumours are unilateral
  • (often right sided) and smaller than 10cm.
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3
Q

What are the diagnostic methods used?

A
  • Urine analysis of vanillymandelic acid (VMA) is often used (false positives may occur e.g. in patients eating vanilla ice cream!)
  • Blood testing for plasma metanephrine levels.
  • CT and MRI scanning are both used to localise the lesion.
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4
Q

Treatment?

A
  • An irreversible alpha adrenoreceptor blocker should be given, although minority may prefer reversible blockade(1).
  • Labetolol may be co-administered for cardiac chronotropic control. Isolated beta blockade should not be considered as it will lead to unopposed alpha activity.
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5
Q

Surgery?

(IVC = inferior vena cava)

A
  • Once medically optimised the phaeochromocytoma should be removed.
  • Most adrenalectomies can now be performed using a laparoscopic approach(2).
  • The adrenals are highly vascular structures and removal can be complicated by catastrophic haemorrhage in the hands of the inexperienced.
  • This is particularly true of right sided resections where the IVC is perilously close.
  • Should the IVC be damaged a laparotomy will be necessary and the defect enclosed within a Satinsky style vascular clamp and the defect closed with prolene sutures.
  • Attempting to interfere with the IVC using any instruments other than vascular clamps will result in vessel trauma and make a bad situation much worse.
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6
Q

What about Incidental adrenal lesions?

A

Adrenal lesions may be identified on CT scanning performed for other reasons(3). Factors suggesting benign disease on CT include(4):

  • Size less than 3cm
  • Homogeneous texture
  • Lipid rich tissue
  • Thin wall to lesion
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