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5 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
MEN1 is an inherited condition, one of the multiple endocrine neoplasia syndromes, in which a faulty MEN1 gene lets tumours grow in the parathyroid glands, the pancreas and the pituitary. Each tumour is treated much as it would be in anyone else, but because there are many of them and they come back, families are followed for life in specialist clinics.
MEN1 is an autosomal dominant disorder caused by germline mutations in the tumour suppressor gene MEN1, which encodes the 610-amino-acid protein menin. It is defined by tumours of the parathyroid glands, the pancreatic islets and the anterior pituitary; some patients also develop carcinoid (neuroendocrine) tumours of the thymus, lung and stomach, adrenocortical tumours, meningiomas, facial angiofibromas, collagenomas and lipomas (Thakker 2012). The WHO classification of endocrine and neuroendocrine tumours (5th edition) lists MEN1 among the genetic tumour syndromes rather than as a tumour, which is why it sits here as an entity under the syndromes page.
How it differs from its parent: MEN1 is the menin-driven syndrome; MEN2 is driven by RET and centres on the thyroid. Within MEN1 the pancreatic and thymic tumours carry the mortality: patients have a decreased life expectancy, and the outcomes of treatments that work in sporadic tumours are less good because the tumours are multiple, often larger and more aggressive, and metastases coincide (Thakker 2012). Thymic neuroendocrine tumour accounts for almost a fifth of MEN1-associated deaths; its pooled prevalence in 2,710 MEN1 patients was 3.7 percent, four fifths of them men (Ye 2017). Parathyroid carcinoma is rare in MEN1: one case in 348 patients (0.28 percent) in a Mayo cohort, with ten reported in the literature (Singh Ospina 2016).
| Setting | Approach | Guideline |
|---|---|---|
| All manifestations | Treated by manifestation as the parent page describes, with germline testing of relatives and lifelong multidisciplinary surveillance. | not mapped |