Multiple endocrine neoplasia

The 8th International Workshop on Multiple Endocrine Neoplasia was held at the Van Andel Institute in Grand Rapids, Michigan, USA from 15–18 June 2002. A total of 180 participants, both clinicians and basic scientists from 18 different countries, attended this meeting. The meeting, which lasted two and a half days, focused on the latest advances in basic science and clinical medicine in the field of endocrine neoplasia syndromes, from the classical MEN types 1 and 2 to other related syndromes. Although the field of MEN1 has witnessed tremendous progress in its genetics and pathology, its clinical management is still confronted with challenges and controversies, especially in relation to pancreatic–duodenal disease, the most frequent cause of mortality and morbidity in MEN1 patients. Here, Doherty and Thompson address the surgical management of MEN1-related pancreatic–duodenal disease. They describe and discuss in great detail the various clinical aspects of the disease, including screening, diagnosis, natural history, operative approaches and follow-up. Based on their and others’ data and experience, they make recommendations for managing this disease. It is noteworthy that besides these problems in the gastrointestinal tract, MEN1 patients may also develop leiomyoma in the oesophagus, as well as in other organs such as lung and uterus [1]. MEN1-related pituitary disease is considered a benign disease and has attracted the least attention amongst the three main MEN-related tumours (parathyroid, endocrine pancreas and anterior pituitary). However, a recent report on a large series of MEN1-related pituitary cases has shown that they are actually more aggressive than their sporadic counterparts [2]. Beckers et al. give an excellent account of the state-of-the-art treatment of MEN1related tumours and compare its application to both hereditary and sporadic cases. The meeting also drew a lot of attention of the participants to the problem of thymic carcinoids in MEN1 as evidenced by the three abstracts presented. The disease occurs predominantly in male MEN1 patients, usually in their thirties and forties. To date, there is no effective treatment and the mortality rate is very high [3]. More molecular studies are warranted to shed light on its biology and hopefully on targets for therapy. Since the cloning of the MEN1 gene, over 300 different mutations have been found in MEN1 patients and sporadic tumours, but to date no clear genotype–phenotype correlation can be demonstrated. In addition, several groups have attempted to elucidate their functions and role in causing the disease. In their article, Chandrasekkarappa and Teh summarize the reported MEN1 mutations, the biological properties of menin and recent reports on menin-interacting proteins. To date, most of these studies, including those on JunD, Smad3 and NF-jB have pointed to transcription and cell growth regulation. In addition, the article also touches on a MEN1 mouse model in which the heterozygous form develops all three main types of tumours. It mimics the human disease and may serve as an appropriate Journal of Internal Medicine 2003; 253: 588–589