{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T18:22:54Z","timestamp":1792174974693,"version":"4.4.0"},"reference-count":79,"publisher":"Bioscientifica","issue":"10","content-domain":{"domain":["erc.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,10,1]]},"abstract":"\n Pituitary neuroendocrine tumours (PitNETs) associated with paragangliomas or phaeochromocytomas are rare.\n SDHx<\/jats:italic>\n variants are estimated to be associated with 0.3\u20131.8% of PitNETs. Only a few case reports have documented the association with\n MAX<\/jats:italic>\n variants. Prolactinomas are the most common PitNETs occurring in patients with\n SDHx<\/jats:italic>\n variants, followed by somatotrophinomas, clinically non-functioning tumours and corticotrophinomas. One pituitary carcinoma has been described.\n SDHC, SDHB<\/jats:italic>\n and\n SDHA<\/jats:italic>\n mutations are inherited in an autosomal dominant fashion and tumorigenesis seems to adhere to Knudson\u2019s two-hit hypothesis.\n SDHD<\/jats:italic>\n and\n SDHAF2<\/jats:italic>\n mutations most commonly have paternal inheritance. Immunohistochemistry for SDHB or MAX and loss of heterozygosity analysis can support the assessment of pathogenicity of the variants. Metabolomics is promising in the diagnosis of\n SDHx<\/jats:italic>\n -related disease. Future research should aim to further clarify the role of\n SDHx<\/jats:italic>\n and\n MAX<\/jats:italic>\n variants or other genes in the molecular pathogenesis of PitNETs, including pseudohypoxic and kinase signalling pathways along with elucidating epigenetic mechanisms to predict tumour behaviour.\n <\/jats:p>","DOI":"10.1530\/erc-22-0157","type":"journal-article","created":{"date-parts":[[2022,8,8]],"date-time":"2022-08-08T09:43:07Z","timestamp":1659951787000},"page":"R157-R172","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-8","source":"Crossref","is-referenced-by-count":27,"title":["Succinate dehydrogenase and MYC-associated factor X mutations in pituitary neuroendocrine tumours"],"prefix":"10.1530","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5491-6730","authenticated-orcid":true,"given":"Paul Benjamin","family":"Loughrey","sequence":"first","affiliation":[{"name":"Patrick G Johnston Centre for Cancer Research, Queen\u2019s University, Belfast, UK"},{"name":"Regional Centre for Endocrinology and Diabetes, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Roncaroli","sequence":"additional","affiliation":[{"name":"Geoffrey Jefferson Brain Research Centre, Division of Neuroscience and Experimental Psychology, School of Medicine, Manchester University, Manchester, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estelle","family":"Healy","sequence":"additional","affiliation":[{"name":"Department of Cellular Pathology, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Weir","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5844-856X","authenticated-orcid":true,"given":"Madhu","family":"Basetti","sequence":"additional","affiliation":[{"name":"Cancer Research UK Cambridge Institute, Li Ka Shing Centre, University of Cambridge, Cambridge, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruth T","family":"Casey","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Cambridge University Hospital NHS Foundation Trust, Cambridge, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven J","family":"Hunter","sequence":"additional","affiliation":[{"name":"Regional Centre for Endocrinology and Diabetes, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4101-9432","authenticated-orcid":true,"given":"M\u00e1rta","family":"Korbonits","sequence":"additional","affiliation":[{"name":"Centre for Endocrinology, William Harvey Research Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Journal of Medical Genetics","doi-asserted-by":"publisher","first-page":"384","DOI":"10.1136\/jmedgenet-2017-105127","article-title":"Tumour risks and genotype-phenotype correlations associated with germline variants in succinate dehydrogenase subunit genes SDHB, SDHC and SDHD","volume":"55","author":"Andrews","year":"2018","unstructured":"Andrews KAAscher DBPires DEVBarnes DRVialard LCasey RTBradshaw NAdlard JAylwin SBrennan P2018Tumour risks and genotype-phenotype correlations associated with germline variants in succinate dehydrogenase subunit genes SDHB, SDHC and SDHD. 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