Bilateral and recurrent adrenocortical carcinoma in MEN1: a case report and review of the literature.

Abstract: The autosomal dominant hereditary cancer syndrome multiple endocrine neoplasia type 1 (MEN1) is associated with an increased prevalence of both benign and malignant adrenal lesions. Although MEN1 mutations are implicated in around 7% of all adrenocortical carcinomas (ACCs), ACC itself is a rare occurrence within the MEN1 syndrome, and the majority of adrenal lesions are benign hyperplastic or adenomatous changes. Differentiating between a benign and malignant adrenal lesion in patients with MEN1 can be challenging, especially in the context of other primary cancers. We report the case of a 76-year-old lady with a genetic diagnosis of MEN1 who presented with a new 20 mm right adrenal nodule and progressive nodularity around the left adrenalectomy surgical bed, 9 years after adrenalectomy for left-sided ACC. Assessment of the right adrenal nodule was challenging in the presence of multiple other primary tumours, including neuroendocrine tumours, a renal lesion of uncertain significance, and a large intramuscular lipomatous lesion. We discuss how molecular imaging methods, including Learning points: MEN1 is associated with an increased incidence of both benign and malignant adrenal lesions.The incidence of ACC among patients with MEN1 is between 0.5 and 6.9%, with a female predisposition.Bilateral ACC is rare in patients with MEN1 and in the wider ACC population.Positron emission tomography (PET) scanning using molecular tracers that bind to steroidogenic enzymes can identify whether lesions are adrenocortical in origin and has an emerging role in the diagnosis of indeterminate adrenal lesions, particularly in the presence of other primary cancers.