Endocrine Abstracts

Multiple Endocrine Neoplasia type 1 (MEN1) is a rare hereditary autosomal dominant disorder characterised by the occurrence of multiple endocrine tumours, predominantly affecting parathyroid glands, pancreatic islet cells and anterior pituitary. Consensus guidelines for MEN1 recommend intensive clinical, biochemical and radiological surveillance commencing in early childhood. The current regimen, which is subject to debate given lack of strong evidence for some aspects of care, includes annual prolactin/IGF-1 with MRI pituitary 3-5 yearly. The aim is to assess current practice for detection and follow up of pituitary abnormalities in our MEN1 cohort. A single-centre retrospective analysis of all MEN1 patients registered in the new dedicated clinic was performed assessing previous compliance with recommendations with review of MRI findings. Twenty-three patients (M:F,1:1.56, Age 44(20-69)years) were included. All had initial MRI pituitary 1-10(median 2) years following diagnosis. Where normal, recommendations for further were followed in three patients. 39% had pituitary adenoma. Six patients had microadenomas; two microprolactinomas which responded well biochemically and radiologically to dopamine agonist(DA). Two patients with normal pituitary on first screening showed microadenoma at 2 year follow up (both non-functioning). Three patients had macroadenoma; one macroprolactinoma with good response to DA for 30 years then proceeded to surgery when tumour progressed, necrosed and compromised vision. Two non-functioning macroadenomas had surgery, one with recurrence/radiotherapy at 7 years. Three patients had hyperprolactinemia with normal MRI. This review based on a small cohort of Northern Ireland MEN1 patients shows pituitary adenomas in 40%, in line with other studies. There is suggestion that macroadenomas may following a more aggressive course here but further analyses are required. These and improving compliance with consensus guidelines are in progress with the streamlining of MEN1 patients to dedicated service.