Multiple endocrine neoplasia (MEN-1) is a rare congenital disease with genetic background. The MEN-1 gene encodes the menin protein, which acts as a tumour suppressor. Mutation of one allele and the inactivation of the other allele of this gene lead to clonal proliferation and to the development of tumours. The clinical manifestation of MEN-1 is a combination of endocrine (most commonly parathyreoid adenomas, entero-pancreatic endocrine tumours, pituitary tumours) and non-endocrine tumours.
In 1993 a 42-year-old primary hyperparathyroidic male patient was operated on an adenoma located in one of his right parathyreoid glands. Six years later, the patient was treated because of repeated gastrointestinal bleedings due to multiple jejunal ulcers. Magnetic resonance imaging (MRI) and octreotide scintigraphy showed a 15 mm tumour in the pancreas, consistent with high level of serum gastrin. A diagnosis of gastrinoma was established and somatostatin analogue (SSA) treatment was started. One year later, primary hyperparathyroidism recurred due to an adenoma in the left parathyroid gland. For this reason, parathyreoidectomy was completed and a quarter of one gland was autotransplanted subcutaneously. Furthermore, increased level of prolactin was measured repeatedly, and sella MRI showed a 3 mm microadenoma in the adenohypophysis. Bromocriptine (Br) medication was then commenced. Under continuous SSA and Br therapy for 10 years, abdominal CT (computer tomography) and MRI check-ups disclosed an arenal incidentaloma whereas no pancreatic tumour could be detected by these techniques. In this report, we present a successful 10-year SSA treatment of a MEN-1 related gastrinoma with the disappearence of its clinical symptoms.However, a temporary withrawal of SSA in the 10 year resulted in clinical relaps and octreoscan revealed the pancreatic tumour again. The disease is now under effective control of further SSA administration.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology