{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,25]],"date-time":"2026-10-25T09:07:42Z","timestamp":1792919262511,"version":"4.5.0"},"reference-count":119,"publisher":"Georg Thieme Verlag KG","issue":"07","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Horm Metab Res"],"published-print":{"date-parts":[[2019,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Pituitary adenomas (PA) and pheochromocytomas\/paragangliomas (PHEO\/PGL) are rare tumors. Although they may co-exist by coincidence, there is mounting evidence that genes predisposing in PHEO\/PGL development, may play a role in pituitary tumorigenesis. In 2012, we described a GH-secreting PA caused by an SDHD mutation in a patient with familial PGLs and found loss of heterozygosity at the SDHD locus in the pituitary tumor, along with increased hypoxia-inducible factor 1\u03b1 (HIF-1\u03b1) levels. Additional patients with PAs and SDHx defects have since been reported. Overall, prevalence of SDHx mutations in PA is very rare (0.3\u20131.8% in unselected cases) but we and others have identified several cases of PAs with PHEOs\/PGLs, like our original report, a condition which we termed the 3\u2009P association (3PAs). Interestingly, when 3PAs is found in the sporadic setting, no SDHx defects were identified, whereas in familial PGLs, SDHx mutations were identified in 62.5\u201375% of the reported cases. Hence, pituitary surveillance is recommended among patients with SDHx defects. It is possible that the SDHx germline mutation-negative 3PAs cases may be due to another gene, epigenetic changes, mutations in modifier genes, mosaicism, somatic mutations, pituitary hyperplasia due to ectopic hypothalamic hormone secretion or a coincidence. PA in 3PAs are mainly macroadenomas, more aggressive, more resistant to somatostatin analogues, and often require surgery. Using the Sdhb\n\n+\/\u2212\n mouse model, we showed that hyperplasia may be the first abnormality in tumorigenesis as initial response to pseudohypoxia. We also propose surveillance  and follow-up approach of patients presenting with this association.<\/jats:p>","DOI":"10.1055\/a-0661-0341","type":"journal-article","created":{"date-parts":[[2018,10,1]],"date-time":"2018-10-01T18:52:50Z","timestamp":1538419970000},"page":"419-436","source":"Crossref","is-referenced-by-count":35,"title":["The 3PAs: An Update on the Association of Pheochromocytomas, Paragangliomas, and Pituitary Tumors"],"prefix":"10.1055","volume":"51","author":[{"given":"Paraskevi","family":"Xekouki","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, King\u2019s College Hospital, London, UK"},{"name":"Division of Diabetes & Nutritional Sciences, King\u2019s College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Brennand","sequence":"additional","affiliation":[{"name":"Division of Diabetes & Nutritional Sciences, King\u2019s College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ben","family":"Whitelaw","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, King\u2019s College Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karel","family":"Pacak","sequence":"additional","affiliation":[{"name":"Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constantine A.","family":"Stratakis","sequence":"additional","affiliation":[{"name":"Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2018,10,1]]},"reference":[{"key":"ref1","volume-title":"WHO Classification of Tumours of Endocrine Organs WHO\/IARC 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