Summary
Background. Multiple endocrine neoplasia (MEN) is an autosomal dominant manner inherited group of diseases characterized by the appearance of tumors in two or more endocrine glands. Tumors of the parathyroid glands, pancreatic is let cells and an te rior pi tu itary gland are char ac ter is tic for MEN-1. The clin i cal manifes tation of MEN-1 depends on the site of the tumor and its secreted hormones. According to the latest 2012 Clinical Practice Guide lines for Multiple Endocrine Neoplasia Type 1, the diagnosis of MEN-1 is based on one of the three criteria. The treatment of each tumor is similar to that found in non-MEN-1. However, it is more complex and requires long-term follow-up.
Material and methods. We are presenting a case of a 36-years-old patient who was accidentally diagnosed with pancreatic neuroendocrine tumor (pNETs) during an abdominal computed tomography (CT) scan. Suspecting MEN-1 other diagnostic tests were performed. Primary hyperparathyroidism and anterior pituitary microadenoma were detected and MEN-1 was diagnosed and later genetically confirmed. Enucleation of pNET and subtotal parathyroidectomy were performed. Anterior pituitary microadenoma was treated with bromocriptine therapy. The following laboratory tests results were improved, however during patient CT follow-up the second pNET was detected. Patient is prepared for resection of the remaining pNET.
Conclusions. Due to the specific nature of MEN-1, full recovery is impossible. There fore, the main clinical goals are to diagnose MEN-1 early, perform genetic testing for the patient and its family members and ensure consistent follow- up after conservative or surgical treatment for prevention of tumor reoccurrence, metastases and targeted organ damage.
