SFEBES2008 Poster Presentations Endocrine tumours and neoplasia (31 abstracts)
1Aintree University Hospitals, Liverpool, UK; 2Royal Liverpool Childrens Hospital, Liverpool, UK; 3Derriford Hospital, Plymouth, UK.
The multiple endocrine neoplasia (MEN) clinic at our hospital was established in 2002 with the aim of providing integrated, comprehensive and expert regional care to patients with MEN syndrome. The clinical team consists of an adult endocrinologist, a paediatric endocrinologist, a clinical geneticist and an endocrine surgeon.
In 2002, 16 patients with MEN type 1 from five different kindred were seen. These patients had previously been attending a combination of general surgical, general endocrine and gastroenterological clinics. The MEN clinic has quickly expanded over the last 5 years and in 2007, 38 gene positive MEN type 1 patients from 13 kindred attend the clinic. The endocrinopathies diagnosed to date are tabulated below.
Endocrinopathy | No of patients | No of surgical procedures |
10 Hyperparathyroid | 25 | 29 |
Pituitary macroadenoma | 9 | 9 |
Pancreatic/foregut tumours | 8 | 11 |
MEN type 1 has prevalence in the general population between 2 and 20 per 100 000 population. To date, there are known to be at least 113 members from the 13 kindred attending the MEN clinic who have yet to be clinically and genetically tested. Each of these carries between a 12.5 and 50% risk of having MEN type 1 depending on their relationship to the 38 patients already identified. Given the prevalence of MEN type 1, there are likely to be other families in the region with MEN type 1 who have yet to be diagnosed. It can, therefore, be expected that there will be many more patients referred to the MEN clinic in the future for screening and follow-up, and this has huge implications for service planning and provision.