Long term experience after subtotal adrenalectomy for multiple endocrine neoplasia type IIa

Abstract

Objective: To evaluate the long-term results after subtotal adrenalectomy in patients with multiple endocrine neoplasia type IIa (MEN IIa). Design: Retrospective study. Setting: University Hospital, Sweden. Subjects: Five patients who underwent partial adrenalectomy between 1985 and 1989. Interventions: Subtotal adrenalectomy with a rim of cortical tissue left in situ. Main outcome measures: Follow up by interview, measurement of cortisol and catecholamine excretion in urine, and cortisol concentration in serum in response to stimulation with ACTH. Results: Three patients took no corticosteroids regularly, but during upper respiratory tract infections, or periods of severe stress they took 25 mg cortisone acetate daily. This is confirmed by their normal values of 24 hour urinary cortisol excretion and subnormal responses to an ACTH-stimulation test. The fourth and fifth patients had low concentrations of endogenous corticosteroids postoperatively, which is being replaced with 25 mg cortisone acetate daily. Postoperatively all five patients had low urinary adrenaline excretion. Conclusion: Subtotal adrenalectomy in patients with MEN IIa resulted in basal endogenous corticosteroids within the reference range in three of five patients. There was no evidence of reduced adrenocortical function with time, nor were there any signs of recurrence of the pheochromocytoma.