Introduction: Life expectancy is decreased in MEN1 patients due to multiple tumors that may be larger, more aggressive, resistant to treatment and with a higher risk of recurrence.
Case report: We report a 59-year-old male patient with family history of endocrine tumors (father- pituitary adenoma, son- primary hyperparathyroidism, daughter- microprolactinoma, primary hyperparathyroidism). He was first referred to our Clinic in 2007 after being incidendally diagnosed with well differentiated pancreatic neuroendocrine tumor (CT-scan performed for nephrolithiasis and repeated renal colic revealed a pancreatic mass that was surgically removed). In 2009 the patient was diagnosed with primary hyperparathyroidism and multinodular goiter for which subtotal parathyroidectomy and total thyroidectomy were performed. He also presented MEN1 associated tumors such as non-functioning left adrenal adenoma, bilateral asymmetric lipomastia and multiple subcutaneous lipomas and conditions associated with calcium disorders: nephrolithiasis, nephrocalcinosis, secondary osteoporosis. Regarding these findings, the patient was diagnosed with MEN1 syndrome and started undergoing periodical follow-up. Between 2009 and 2016, at the anual reevaluation, no clinical or paraclinical evidence of tumor recurrence was found. In October 2016 he presented a mild elevation of calcium and PTH levels and the sestamibi parathyroid scan revealed the presence of parathyroid adenoma. The CT-scan showed two pancreatic masses, immunohistochemistry tests from the biopsy supported the diagnosis of neuroendocrine tumor NET G1 and the patient started treatment with somatostatin analogues. Further assessment of the possibility of tumor resection and parathyroidectomy is considered.
Conclusion: Considering the high risk of tumor recurrence in MEN1 patients, regular follow-up is crucial in detecting and treating the relapses. Our patient developed both recurrence of neuroendocrine tumor and hyperparathyroidism.
20 - 23 May 2017
European Society of Endocrinology