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Endocrine Abstracts (2018) 56 S17.3 | DOI: 10.1530/endoabs.56.S17.3

ECE2018 Symposia Recent advances in Primary Adrenal Macronodular Hyperplasia (3 abstracts)

Diagnosis and Management of Primary Adrenal Macronodular Hyperplasia

Dimitra Argyro Vassiliadi


Greece.


Macronodular adrenal hyperplasia refers to adrenal enlargement by large nodules that may be related to ACTH-dependent or ACTH-independent pathologies. The term Primary Macronodular Adrenal Hyperplasia (PMAH) was recently introduced to replace the term ACTH-independent macronodular adrenal hyperplasia, since in some cases paracrine ACTH production may contribute to cortisol secretion, and also to encompass cases of unilateral macronodular hyperplasia. PMAH is a heterogeneous disease comprising different entities. The diagnostic approach depends on the clinical context. PMAH is rarely detected as part of the evaluation for ACTH-independent Cushing’s syndrome. It is more often detected incidentally; in this setting imaging and hormonal characterisation are required. According to the recent ESE and ENS@T guidelines imaging characterisation should be done separately for each lesion since occasionally co-occurrence of different entities, such as adenoma, pheochromocytoma, cyst, myelolipoma or even adrenocortical carcinoma, may be encountered. The most common hormonal alteration is autonomous cortisol secretion (ACS). ACS follows a continuum and is best assessed using the 1-mg overnight dexamethasone suppression test. According to recent guidelines values of ≤50 nmol/l (1.8 μg/dl) exclude ‘ACS’, values of >140 nmol/l (5 μg/dl) confirm ‘ACS’ and values between 51 and 140 nmol/l (1.9–5.0 μg/dl) indicate ‘possible ACS’. Additional tests including ACTH levels, midnight cortisol or 24-hr urinary free cortisol may aid in establishing the degree of cortisol excess. In addition, serum 17-hydroxyprogesterone should be measured to exclude congenital adrenal hyperplasia, keeping in mind that increased levels may also represent secretion of steroid precursors from the lesion(s). Testing for adrenal insufficiency may be relevant in some cases (i.e. when imaging suggests bilateral infiltrative disease or haemorrhage). In several occasions, aberrant responses due to illegitimate receptor expression are also encountered by relevant testing. Genetic testing may be offered in selected cases based on recent findings that PMAH is genetically determined. The appropriate management of PMAH remains controversial. Bilateral adrenalectomy is a debilitating option, resulting in lifetime steroid dependency. This is why the recent guidelines suggest against bilateral adrenalectomy in patients with no clinical signs of overt Cushing’s syndrome. In selected patients unilateral adrenalectomy of the dominant lesion or based on adrenal vein sampling results might be considered. The decision for surgery depends on the degree of hypercortisolism, the presence of co-morbidities, age, general health and patient’s preference. In cases where the regulation of cortisol secretion is mediated by aberrant hormone receptors there is some potential for medical therapy.

Volume 56

20th European Congress of Endocrinology

Barcelona, Spain
19 May 2018 - 22 May 2018

European Society of Endocrinology 

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