Presence of Adrenal Tumors in MEN1

Anatomy of the adrenal gland

Multiple endocrine neoplasia type 1 (MEN1) can involve the adrenal glands, but there is limited data on this. Therefore, using a large cohort, French researchers looked at MEN1-associated adrenal lesions; their objective was to provide a rationale for adrenal-involvement management in MEN1.

The study was published in European Journal of Endocrinology. The article, "Adrenal involvement in MEN1", provides an analysis of 715 cases from the Groupe D’Etude des Tumeurs Endocrines database.”

There were 715 patients in the study of MEN1 who were found by searching a multicenter database between 1956 and 2008. These records were analyzed including those of 144 patients with adrenal sporadic incidentalomas (tumors).  The records of patients with adrenal lesions were compared to the records of these adrenal patients with sporadic incidentalomas.

About 20% of the MEN1 patients had adrenal enlargements.  Among those cases, 58.1% were determined to be adrenal tumors (size > 10 mm) that represents 10.1% of the whole patient cohort.  In 12.5% of the adrenal tumor cases, the tumors were bilateral.  Tumors larger than 40 mm were seen in 19.4% of the adrenal tumor cases.

Hormonal hypersecretion was seen only in tumor patients, and it was present in only 15.3% of them.

Comparison of adrenal sporadic incidentaloma and adrenal lesions revealed several interesting results.  MEN1-related tumors involved more cases of primary hyperaldosteronism, fewer pheochromocytomoas, and more adrenocortical carcinomas (ACCs—13.8% in MEN-related tumors vs 1.3% in adrenal sporadic incidentaloma).

There were 10 ACCs in 8 patients, and 9 of the tumors were considered stage I or stage II following the criteria from the European Network for the Study of Adrenal Tumors.  The researchers pointed out that in 2 of the 8 patients, ACCs developed after several years of follow-up on small adrenal tumors.

A genotype/phenotype correlation could not be found for the occurrence of ACC, adrenal lesions, or hormonal hypersecretion.

The pathology of MEN1-related adrenal tumors and adrenal sporadic incidentaloms is different.  If there are no relevant symptoms, endocrine biology should be restricted to adrenal tumor patients, and there should be a focus on steroid secretion, including in the aldosterone-renin system.  Since MEN1 is a high-risk condition for ACCs, that should be taken into consideration if there is an adrenal tumor present, regardless of the size.

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