Introduction: The syndrome of multiple endocrine neoplasia type 1 (MEN 1) is a relatively rare hereditary disease predisposing to a variety of tumours, some of which may be hormonally active. The main organs affected are the parathyroid, the pituitary and the pancreatoduodenal area. Despite the rarity of MEN 1, its occurrence is not negligible in clinical practice and the clinicians ignorance often leads to delayed diagnosis and inadequate treatment.
Material and methods: The aim of this review is to present contemporary literature on this issue, including the most controversial topic, i.e. recent modalities of treatment of pancreatoduodenal tumours, and to present a case report. The literature review was based on computer searches in PubMed and DynaMed.
Discussion: Pancreatoduodenal tumours associated with MEN 1 are relatively slow-growing neoplasias. The development of liver metastases is the main factor of long-term survival. The aim of surgical treatment is the stabilisation of the disease and the prevention of liver metastases. Radical surgical methods may lead to better biochemical curability. The extent of surgical intervention should be selected individually. We discuss our case report in the context of these findings.
Conclusion: Knowledge of the MEN 1 syndrome has its importance in the clinical practice. Further prospective studies will be needed to evaluate the effectiveness of treatment, especially for pancreatoduodenal tumours.