Adrenal incidentaloma, breast cancer and unrecognized multiple endocrine neoplasia type 1

Background. The incidence of adrenal incidentaloma has been increasing proportional to the use of radiologic examination. Multiple endocrine neoplasia1 (MEN1) syndrome may present with various tumors. The present study reports a case of adrenal incidentaloma with unrecognised MEN1 syndrome associated with breast cancer. Clinical case. A 48-year-old woman presented with a 2.4cm left adrenal incidentaloma on abdominal computed tomography. Her history revealed primary amenorrhea, recurrent peptic ulcer and nephrolithiasis. Laboratory and radiologic examination revealed two pancreatic tail mass lesions with markedly elevated gastrin levels (1462 pg/mL), hypercalcemia with increased parathyroid hormone levels (72 pg/mL), a 1.5cm pituitary mass with hyperprolactinemia (234 ng/mL), a 1.0cm meningioma and a nonfunctional left adrenal mass. During this image work up, a 0.6cm nodule in the right breast was incidentally detected. Surgeries (laparoscopic distal pancreatectomy, parathyroidectomy and wide local excision of breast) and pathologic findings confirmed pancreatic neuroendocrine tumors, parathyroid gland hyperplasia, and breast cancer. Carbergoline treatment for 12 months decreased prolactin levels to 27 ng/mL. Genetic testing using peripheral blood revealed a pathogenic variant in MEN1 on chr11q13 (NM_000244.3:c.1365+1_1365+11 del, GTGAGGGACAG, heterozygous). Conclusion. Considering the increasing incidence of adrenal incidentaloma and 20% prevalence of adrenal tumors in patients with MEN1, it is important to rule out MEN1 association in patients with adrenal incidentaloma. Additionally, breast cancer was detected during MEN1 work-up in this case. Female patients with MEN1 are at increased risk for breast cancer. Therefore, intensified breast cancer screening at a relatively young age should be considered in female MEN1 patients.