Endocrine Abstracts

Introduction: MEN1 is a rare syndrome characterised by hyperplasia or neoplasm of the parathyroid glands, pituitary, pancreas or duodenum and can associate, less frequently, phaeochromocytoma, thymic or bronchial carcinoids, multiple lipomas, cutaneous angiofibromas and thyroid adenomas.

Case report: A 47-year-old woman with MEN1 presenting parathyroid recurrent adenomas, a pituitary prolactin-growth hormone cosecreting macroadenoma, associated with an incidentall papillary thyroid microcarcinoma. The patient had a primary hyperparathyroidism treated 12 years ago, diagnosed after she was treated for a coraliform lithiasis. 2 years after, a secondary amenorrhea followed, caused by pituitary prolactin secreting macroadenoma, and a dopamine agonist was started. She didn’t present for the follow ups for 10 years, when another kidney stone was discovered. Laboratory results show hypercalcaemia and increased PTH, with normal TSH and free thyroxine levels. She had a total thyroidectomy and parathyroidectomy. Besides the two parathyroidian adenomas, the histopathological examination revealed a papillary thyroid microcarcinoma. 1 year after, she was admitted in our service. Having a MEN1 patient, we had to continue the investigation. We found a slightly increased PTH level with normal calcaemia most probably secondary to the vitamin D insufficiency. The pituitary check-up showed a 1.01/1.03 cm adenoma with increased prolactin level and surprisingly increased IGF1 levels. Additionally, the patient had no signs or symptoms suggesting a pancreatic-duodenal involvement (no hypoglycaemia, insulin and gastrin in normal range). The next steps are the genetic analysis for MEN1 gene and the treatment for the pituitary macroadenoma.

Conclusion: This case underline the different phenotypic presentation of MEN1. Our patient had the classical presentation hypercalcaemia but she doesn’t associate duodenoenteropancreatic NETs, the second most common endocrine manifestation in MEN1 syndrome. Additionally, the pituitary adenoma cosecrete prolactin and growth hormone. As we found in literature, the papillary thyroid carcinoma is probably incidental.