Natural course of small adrenal lesions in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study

Abstract

Objective: Adrenal lesion is one of the features of multiple endocrine neoplasia type 1 (MEN1). This study aimed to assess prevalence, natural course and clinical relevance of small adrenal lesions without clinical symptoms, endocrine activity, or mechanical problems and thus without clear indication for surgical therapy by endoscopic ultrasound (EUS). Design and methods: Forty-nine patients with familial MEN1 were studied. Twenty-seven of these with adrenal lesions were detected by EUS and at least two performed EUS examinations were included into a subgroup where changes in adrenal morphology were studied by measuring changes in the largest diameter of the dominant adrenal tumour. Results: EUS detected adrenal lesions in 36 (73%) patients: 6 (12%) plump adrenals, 17 (35%) nodular hyperplasia, 12 (24%) adenomas and 1 (2%) cyst. Bilateral adrenal lesions were detected in 17 patients and unilateral in 19 patients. A change in the largest tumour diameter was found to be for nodular hyperplasia K0.02G1.41% per month (range K2.56 to 4.58%) and for adenomas K0.61G1.95% per month (range K6.25 to 1.15%). One patient had an adrenal cyst with significant growth. There was no evidence of carcinoma or metastatic disease during the study. Conclusions: The prevalence of adrenal lesions in MEN1 is higher than that reported earlier. Except one cystic lesion, no significant change in the tumour size was observed over a mean observation period of more than 2 years. In a typical situation, small adrenal lesions in MEN1 seem to be constant in their morphology.European Journal of Endocrinology 158 699-704

Multiple endocrine neoplasia type 1 (MEN1)

“…[37][38][39][40][41] Adrenal adenomas, hyperplasia and cysts are all encountered while adrenal carcinoma in rare in MEN1 (<1%). The frequency of diagnosed adrenal lesions has generally increased over the last two decades and in part reflects more sensitive imaging techniques; indeed, the frequency of incidental adrenal lesions in the general population is not insignificant (up to 6%).…”

The future: medical advances in MEN1 therapeutic approaches and management strategies

“…The incidence of adrenal involvement varies from 5% in early series to 73% in more recent studies (Trump et al 1997, Carty et al 1998, Langer et al 2002, Waldmann et al 2007, Schaefer et al 2008, Pieterman et al 2009, Gatta-Cherifi et al 2012. The majority of adrenal lesions are non-functional and include cortical adenomas, hyperplasia, multiple adenomas, nodular hyperplasia or cysts.…”

“…The majority of adrenal lesions are non-functional and include cortical adenomas, hyperplasia, multiple adenomas, nodular hyperplasia or cysts. Bilateral hyperplasia is also commonly described (Schaefer et al 2008, Gatta-Cherifi et al 2012.…”

“…ACTH-independent Cushing's syndrome and primary hyperaldosteronism are the most encountered clinical syndrome in the presence of an adrenal lesion and cortical hyperfunction (Waldmann et al 2007, Schaefer et al 2008, Gatta-Cherifi et al 2012. Pheochromocytomas are reported in patients with MEN1, but remain rare (Waldmann et al 2007, Schaefer et al 2008.…”

Genetic aspects of adrenocortical tumours and hyperplasias

“…20 Adrenal involvement in MEN1 has been reported in 20-40% of cases; however, endoscopic ultrasound detected adrenal lesions in up to 73% of MEN1 patients. 21 There is a broad range of histological features in MEN1 patients with adrenal involvement, such as uni-or bi-lateral hyperplasia, benign adenoma, and, less frequently, ACC; most of these cases are nonfunctional and exhibit an indolent clinical course. In addition, pheochromocytomas were found in less than 1% of MEN1 patients.…”