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

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 20mm 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 [ 18 F]CETO PET-CT assisted in making the diagnosis of suspected bilateral metachronous ACC with recurrence at her left adrenalectomy site. We review the cohort studies and case reports in the literature to discuss the association between MEN1 and ACC. Learning points