Adrenal venous sampling in patients with ACTH-independent hypercortisolism

Eleni Papakokkinou,Hugo Jakobsson,Gudmundur Johannsson,Augustinas Sakinis,Olof Ehn,Bo Wängberg,Oskar Ragnarsson,Andreas Muth

doi:10.1007/s12020-019-02038-0

Eleni Papakokkinou, Hugo Jakobsson + Show 6 more

Open Access

https://doi.org/10.1007/s12020-019-02038-0

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Abstract

PurposeTo study the usefulness of adrenal venous sampling (AVS) in distinguishing unilateral from bilateral cortisol production in patients with ACTH-independent hypercortisolism and bilateral adrenal lesions, or morphologically normal adrenal glands.MethodsA retrospective analysis of ten consecutive patients with ACTH-independent hypercortisolism who underwent AVS at our institution between 2009 and 2017. Unilateral dominant cortisol production was defined as a side-to-side cortisol/aldosterone lateralization ratio >2.ResultsFour of ten patients had overt Cushing’s syndrome. Of these, two had bilateral adrenal lesions on computed tomography and two had normal adrenal glands. One of the two patients with bilateral adrenal lesions had, based on the AVS, a unilateral dominant cortisol production. Following unilateral adrenalectomy the patient developed adrenal insufficiency. The other three patients were considered to have bilateral cortisol production and underwent bilateral adrenalectomy. Six patients had a mild autonomous cortisol secretion and bilateral adrenal lesions. Based on AVS, one patient was considered to have unilateral dominant cortisol production, underwent unilateral adrenalectomy and developed transient adrenal insufficiency postoperatively.ConclusionsAVS may contribute to appropriate treatment in patients with ACTH-independent hypercortisolism and bilateral adrenal lesions. In our series, AVS was helpful in the decision-making of two out of ten patients, avoiding chronic treatment with steroidogenesis inhibitors, or inappropriate bilateral adrenalectomy.

Highlights

Adrenal Cushing’s syndrome (CS) is characterized by autonomous cortisol production with low plasma ACTH concentrations and is defined as ACTHindependent CS
In our cohort, which included patients with ACTHindependent hypercortisolism and bilateral adrenal lesions or normal adrenal glands, adrenal venous sampling (AVS) contributed to an appropriate choice of unilateral adrenalectomy in two out of ten patients
Unilateral minimally invasive adrenalectomy is the primary treatment of choice for patients with CS caused by unilateral cortisol producing adrenal adenomas

Summary

Introduction

Adrenal Cushing’s syndrome (CS) is characterized by autonomous cortisol production with low plasma ACTH concentrations and is defined as ACTHindependent CS. ACTH-independent CS accounts for 15–20% of endogenous CS where the most common cause is a unilateral cortisol producing adrenal adenoma [1]. 10% of patients with ACTH-independent CS have bilateral adrenal lesions [3]. Mild autonomous cortisol secretion (MACS) is defined as a mild hypersecretion of cortisol in patients with adrenal incidentaloma but without the typical signs and symptoms of clinically overt hypercortisolism. 9–17% of patients with adrenal incidentalomas have bilateral lesions [5, 6], and seem to have an even higher prevalence of MACS than patients with unilateral lesions [7]

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