Loading
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).
Treatment is by manifestation, as the parent page sets out: parathyroid surgery for hyperparathyroidism, resection of functioning or larger pancreatic neuroendocrine tumours, medical treatment of gastrinoma, and the sporadic pathways for pituitary and neuroendocrine tumours. The guideline recommends presymptomatic detection by MEN1 mutation testing of first-degree relatives and lifelong surveillance under a multidisciplinary team with experience of endocrine tumours (Thakker 2012). Menin inhibitors approved for leukaemia act on the same protein but have no trial in MEN1.
Orphanet lists MEN1 as a rare disease (ORPHA:652). The 2012 international guideline gives no incidence figure in its abstract; first-degree relatives of a carrier have a 50 percent chance of inheriting the mutation (Thakker 2012). No GLOBOCAN estimate exists for a syndrome.
Site decides cause and behaviour: HPV drives oropharyngeal cancer, EBV drives nasopharyngeal cancer, tobacco drives oral and laryngeal cancer; all drain into the neck node levels that surgeons and radiotherapists map.
Same organ: Acinic cell carcinoma of the salivary glands, Carcinoma ex pleomorphic adenoma, Multiple endocrine neoplasia type 2 (MEN2A and MEN2B), Hyperparathyroidism-jaw tumour syndrome (CDC73-related parathyroid carcinoma), Oropharyngeal cancer (tonsil and base of tongue), Laryngeal and hypopharyngeal cancer, Oral cavity cancer (mouth and tongue), Head and neck squamous cell carcinoma, Nasopharyngeal carcinoma, Salivary gland cancers, Papillary thyroid cancer, Follicular thyroid cancer, Medullary thyroid cancer, Anaplastic thyroid cancer, Thyroid cancer, Nasal cavity and paranasal sinus cancers (including esthesioneuroblastoma), NUT carcinoma (midline carcinoma with NUTM1 rearrangement), Parathyroid carcinoma, Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4), HPV-positive oropharyngeal cancer, HPV-negative head and neck squamous cell carcinoma (including HPV-negative oropharyngeal cancer), Recurrent or metastatic head and neck squamous cell carcinoma, Hypopharyngeal cancer, Adenoid cystic carcinoma, Salivary duct carcinoma, Mucoepidermoid carcinoma, Oral tongue and floor of mouth cancer, Buccal mucosa and gingivobuccal cancer (oral cancer in India), Lip cancer, Locoregionally advanced nasopharyngeal carcinoma (stage III to IVA), Recurrent and metastatic nasopharyngeal carcinoma, Esthesioneuroblastoma (olfactory neuroblastoma), Sinonasal undifferentiated carcinoma (SNUC) and SWI/SNF-deficient sinonasal carcinoma
Treated by manifestation as the parent page describes, with germline testing of relatives and lifelong multidisciplinary surveillance.
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Query for this cancer: (TITLE:"Multiple endocrine neoplasia type 1" OR ABSTRACT:"Multiple endocrine neoplasia type 1" OR TITLE:"MEN1" OR ABSTRACT:"MEN1" OR TITLE:"Wermer syndrome" OR ABSTRACT:"Wermer syndrome" OR TITLE:"MEN1 menin; parathyroid, pancreatic NET, pituitary" OR ABSTRACT:"MEN1 menin; parathyroid, pancreatic NET, pituitary") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Multiple endocrine neoplasia type 1 (MEN1), not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Newly diagnosed? Read the first 60 days with Multiple endocrine neoplasia type 1, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.