{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T11:14:34Z","timestamp":1780398874077,"version":"3.54.1"},"reference-count":90,"publisher":"Bioscientifica","issue":"2","funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["T32DK007028"],"award-info":[{"award-number":["T32DK007028"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["erc.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2026,2,12]]},"abstract":"<jats:p>\n                    Multiple endocrine neoplasia type 5 (MEN5) is a newly proposed hereditary syndrome caused by heterozygous germline loss-of-function mutations in the\n                    <jats:italic>MAX<\/jats:italic>\n                    gene, which encodes MYC-associated factor X (MAX). Initially only linked to pheochromocytoma\/paraganglioma (PCC\/PGL) syndrome,\n                    <jats:italic>MAX<\/jats:italic>\n                    mutations have also been associated with other tumors. This review synthesizes current knowledge on the discovery, pathogenesis, clinical features, diagnosis, and management of MEN5. The pathogenesis of MEN5 involves disruption of tumor suppressor MAX\u2013MXD heterodimers in the context of MAX loss, leading to increased cell proliferation. Tumor development in MAX-associated disease is consistent with the two-hit mechanism, in which a heterozygous germline mutation is followed by somatic inactivation of the remaining allele. Clinically, MEN5 is characterized by a diverse tumor spectrum, with PCC\/PGL and pituitary adenoma being the most common manifestation. Other reported conditions include primary hyperparathyroidism, renal oncocytoma, and neuroblastoma, but the connection between the mutation and development of some of these tumors has not been definitively proven. Evidence from published reports suggests a parent-of-origin effect, with higher penetrance more commonly observed when the pathogenic variant is paternally inherited. Despite recent progress, the epidemiology, penetrance, and genotype\u2013phenotype associations of MEN5 require further investigation. Taken together, MEN5 represents a novel hereditary endocrine neoplasia syndrome with a broader tumor spectrum than initially suspected. The\n                    <jats:italic>MAX<\/jats:italic>\n                    gene is included in several diagnostic gene panels, and endocrinologists should be aware of the associated disorders. The limitations of current knowledge highlight the need for further research to better characterize its clinical features, pathogenesis, and management.\n                  <\/jats:p>","DOI":"10.1530\/erc-25-0219","type":"journal-article","created":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T10:42:28Z","timestamp":1770979348000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-8","source":"Crossref","is-referenced-by-count":1,"title":["Multiple endocrine neoplasia type 5: emerging evidence and clinical perspectives"],"prefix":"10.1530","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6501-6180","authenticated-orcid":true,"given":"Nipith","family":"Charoenngam","sequence":"first","affiliation":[{"name":"Endocrine Unit, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0867-9778","authenticated-orcid":true,"given":"Michael","family":"Mannstadt","sequence":"additional","affiliation":[{"name":"Endocrine Unit, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Cancer Res","first-page":"3119","article-title":"MAX and MYC: a heritable breakup","volume":"72","author":"Casc\u00f3n A","year":"2012","unstructured":"Casc\u00f3n A\n & \nRobledo M\n\n. MAX and MYC: a heritable breakup. Cancer Res 2012 72 3119\u20133124. (https:\/\/doi.org\/10.1158\/0008-5472.CAN-11-3891)"},{"key":"bib2","series-title":"Nat Genet","first-page":"663","article-title":"Exome sequencing identifies MAX mutations as a cause of hereditary pheochromocytoma","volume":"43","author":"Comino-M\u00e9ndez I","year":"2011","unstructured":"Comino-M\u00e9ndez I\n, \nGracia-Azn\u00e1rez FJ\n, \nSchiavi F\n, et al.\n Exome sequencing identifies MAX mutations as a cause of hereditary pheochromocytoma. Nat Genet 2011 43 663\u2013667. (https:\/\/doi.org\/10.1038\/ng.861)"},{"key":"bib3","series-title":"Clin Cancer Res","first-page":"2828","article-title":"MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma","volume":"18","author":"Burnichon N","year":"2012","unstructured":"Burnichon N\n, \nCasc\u00f3n A\n, \nSchiavi F\n, et al.\n MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma. Clin Cancer Res 2012 18 2828\u20132837. (https:\/\/doi.org\/10.1158\/1078-0432.ccr-12-0160)"},{"key":"bib4","series-title":"J Clin Endocrinol Metab","first-page":"e1163","article-title":"Multiple endocrine tumors associated with germline MAX mutations: multiple endocrine neoplasia type 5?","volume":"106","author":"Seabrook AJ","year":"2021","unstructured":"Seabrook AJ\n, \nHarris JE\n, \nVelosa SB\n, et al.\n Multiple endocrine tumors associated with germline MAX mutations: multiple endocrine neoplasia type 5? J Clin Endocrinol Metab 2021 106 e1163\u2013e1182. (https:\/\/doi.org\/10.1210\/clinem\/dgaa957)"},{"key":"bib5","series-title":"Pituitary","first-page":"127","article-title":"Loss of heterozygosity and absence of MAX immunostaining in a prolactinoma associated with multiple endocrine neoplasia type 5 (MEN5)","volume":"28","author":"Delemer B","year":"2025","unstructured":"Delemer B\n, \nFlorea SM\n, \nDecoudier B\n, et al.\n Loss of heterozygosity and absence of MAX immunostaining in a prolactinoma associated with multiple endocrine neoplasia type 5 (MEN5). Pituitary 2025 28 127. (https:\/\/doi.org\/10.1007\/s11102-025-01607-z)"},{"key":"bib6","series-title":"Front Endocrinol","article-title":"Case report: pheochromocytoma and synchronous neuroblastoma in a family with hereditary pheochromocytoma associated with a MAX deleterious variant","volume":"12","author":"Duarte DB","year":"2021","unstructured":"Duarte DB\n, \nFerreira L\n, \nSantos AP\n, et al.\n Case report: pheochromocytoma and synchronous neuroblastoma in a family with hereditary pheochromocytoma associated with a MAX deleterious variant. Front Endocrinol 2021 12 609263. (https:\/\/doi.org\/10.3389\/fendo.2021.609263)"},{"key":"bib7","series-title":"J Clin Endocrinol Metab","first-page":"4013","article-title":"Clinical and molecular features of renal and pheochromocytoma\/paraganglioma tumor association syndrome (RAPTAS): case series and literature review","volume":"102","author":"Casey RT","year":"2017","unstructured":"Casey RT\n, \nWarren AY\n, \nMartin JE\n, et al.\n Clinical and molecular features of renal and pheochromocytoma\/paraganglioma tumor association syndrome (RAPTAS): case series and literature review. J Clin Endocrinol Metab 2017 102 4013\u20134022. (https:\/\/doi.org\/10.1210\/jc.2017-00562)"},{"key":"bib8","series-title":"J Clin Endocrinol Metab","first-page":"453","article-title":"Complex MAX rearrangement in a family with malignant pheochromocytoma, renal oncocytoma, and erythrocytosis","volume":"101","author":"Korpershoek E","year":"2016","unstructured":"Korpershoek E\n, \nKoffy D\n, \nEussen BH\n, et al.\n Complex MAX rearrangement in a family with malignant pheochromocytoma, renal oncocytoma, and erythrocytosis. J Clin Endocrinol Metab 2016 101 453\u2013460. (https:\/\/doi.org\/10.1210\/jc.2015-2592)"},{"key":"bib9","series-title":"Horm Metab Res","first-page":"784","article-title":"Pancreatic neuroendocrine neoplasm associated with a familial MAX deletion","volume":"52","author":"Petignot S","year":"2020","unstructured":"Petignot S\n, \nDaly AF\n, \nCastermans E\n, et al.\n Pancreatic neuroendocrine neoplasm associated with a familial MAX deletion. Horm Metab Res 2020 52 784\u2013787. (https:\/\/doi.org\/10.1055\/a-1186-0790)"},{"key":"bib10","series-title":"Endocrine","first-page":"701","article-title":"Expanding the phenotype of multiple endocrine neoplasia type 5 (MEN5): pituitary gigantism, myelolipoma and familial pheochromocytoma due to a germline pathogenic MAX variant","volume":"88","author":"Haider A","year":"2025","unstructured":"Haider A\n, \nSundar J\n, \nBeckers A\n, et al.\n Expanding the phenotype of multiple endocrine neoplasia type 5 (MEN5): pituitary gigantism, myelolipoma and familial pheochromocytoma due to a germline pathogenic MAX variant. Endocrine 2025 88 701\u2013705. (https:\/\/doi.org\/10.1007\/s12020-025-04186-y)"},{"key":"bib11","series-title":"JCEM Case Rep","article-title":"Early-onset sarcoma with germline MAX variant: expanding the spectrum in hereditary pheochromocytoma and paraganglioma","volume":"3","author":"Eren A","year":"2025","unstructured":"Eren A\n, \nBrock PL\n & \nDedhia PH\n\n. Early-onset sarcoma with germline MAX variant: expanding the spectrum in hereditary pheochromocytoma and paraganglioma. JCEM Case Rep 2025 3 luaf119. (https:\/\/doi.org\/10.1210\/jcemcr\/luaf119)"},{"key":"bib12","series-title":"Cell","first-page":"193","article-title":"X-Ray structures of Myc-Max and Mad-Max recognizing DNA: molecular bases of regulation by proto-oncogenic transcription factors","volume":"112","author":"Nair SK","year":"2003","unstructured":"Nair SK\n & \nBurley SK\n\n. X-Ray structures of Myc-Max and Mad-Max recognizing DNA: molecular bases of regulation by proto-oncogenic transcription factors. Cell 2003 112 193\u2013205. (https:\/\/doi.org\/10.1016\/s0092-8674(02)01284-9)"},{"key":"bib13","series-title":"Annu Rev Cell Dev Biol","first-page":"653","article-title":"The Myc\/Max\/Mad network and the transcriptional control of cell behavior","volume":"16","author":"Grandori C","year":"2000","unstructured":"Grandori C\n, \nCowley SM\n, \nJames LP\n, et al.\n The Myc\/Max\/Mad network and the transcriptional control of cell behavior. Annu Rev Cell Dev Biol 2000 16 653\u2013699. (https:\/\/doi.org\/10.1146\/annurev.cellbio.16.1.653)"},{"key":"bib14","series-title":"Genes Dev","first-page":"17","article-title":"Essential role for Max in early embryonic growth and development","volume":"14","author":"Shen-Li H","year":"2000","unstructured":"Shen-Li H\n, \nO\u2019Hagan RC\n, \nHou H Jr\n, et al.\n Essential role for Max in early embryonic growth and development. Genes Dev 2000 14 17\u201322. (https:\/\/doi.org\/10.1101\/gad.14.1.17)"},{"key":"bib15","series-title":"Front Med","first-page":"412","article-title":"The MYC transcription factor network: balancing metabolism, proliferation and oncogenesis","volume":"12","author":"Carroll PA","year":"2018","unstructured":"Carroll PA\n, \nFreie BW\n, \nMathsyaraja H\n, et al.\n The MYC transcription factor network: balancing metabolism, proliferation and oncogenesis. Front Med 2018 12 412\u2013425. (https:\/\/doi.org\/10.1007\/s11684-018-0650-z)"},{"key":"bib16","series-title":"Blood","first-page":"1019","article-title":"Development and survival of MYC-driven lymphomas require the MYC antagonist MNT to curb MYC-induced apoptosis","volume":"135","author":"Nguyen HV","year":"2020","unstructured":"Nguyen HV\n, \nVandenberg CJ\n, \nNg AP\n, et al.\n Development and survival of MYC-driven lymphomas require the MYC antagonist MNT to curb MYC-induced apoptosis. Blood 2020 135 1019\u20131031. (https:\/\/doi.org\/10.1182\/blood.2019003014)"},{"key":"bib17","series-title":"J Biol Chem","first-page":"2001","article-title":"The MNT transcription factor autoregulates its expression and supports proliferation in MYC-associated factor X (MAX)-deficient cells","volume":"295","author":"Lafita-Navarro MC","year":"2020","unstructured":"Lafita-Navarro MC\n, \nLia\u00f1o-Pons J\n, \nQuintanilla A\n, et al.\n The MNT transcription factor autoregulates its expression and supports proliferation in MYC-associated factor X (MAX)-deficient cells. J Biol Chem 2020 295 2001\u20132017. (https:\/\/doi.org\/10.1074\/jbc.ra119.010389)"},{"key":"bib18","series-title":"Cancer Res","first-page":"5565","article-title":"Mnt-deficient mammary glands exhibit impaired involution and tumors with characteristics of Myc overexpression","volume":"66","author":"Toyo-oka K","year":"2006","unstructured":"Toyo-oka K\n, \nBowen TJ\n, \nHirotsune S\n, et al.\n Mnt-deficient mammary glands exhibit impaired involution and tumors with characteristics of Myc overexpression. Cancer Res 2006 66 5565\u20135573. (https:\/\/doi.org\/10.1158\/0008-5472.can-05-2683)"},{"key":"bib19","series-title":"Proc Natl Acad Sci U S A","first-page":"6912","article-title":"Glucose sensing by MondoA:Mlx complexes: a role for hexokinases and direct regulation of thioredoxin-interacting protein expression","volume":"105","author":"Stoltzman CA","year":"2008","unstructured":"Stoltzman CA\n, \nPeterson CW\n, \nBreen KT\n, et al.\n Glucose sensing by MondoA:Mlx complexes: a role for hexokinases and direct regulation of thioredoxin-interacting protein expression. Proc Natl Acad Sci U S A 2008 105 6912\u20136917. (https:\/\/doi.org\/10.1073\/pnas.0712199105)"},{"key":"bib20","series-title":"The Myc\/Max\/Mad Transcription Factor Network","first-page":"255","article-title":"The mlx network: evidence for a parallel Max-like transcriptional network that regulates energy metabolism","author":"Billin AN","year":"2006","unstructured":"Billin AN\n & \nAyer DE\n\n. The mlx network: evidence for a parallel Max-like transcriptional network that regulates energy metabolism. In The Myc\/Max\/Mad Transcription Factor Network, pp 255\u2013278. Ed \n\nRN Eisenman\n\n. Berlin, Heidelberg, Germany: Springer, 2006. (https:\/\/doi.org\/10.1007\/3-540-32952-8_10)"},{"key":"bib21","series-title":"Sci Adv","article-title":"MAX inactivation deregulates the MYC network and induces neuroendocrine neoplasia in multiple tissues","volume":"11","author":"Freie B","year":"2025","unstructured":"Freie B\n, \nIbrahim AH\n, \nCarroll PA\n, et al\n. MAX inactivation deregulates the MYC network and induces neuroendocrine neoplasia in multiple tissues. Sci Adv 2025 11 eadt3177. (https:\/\/doi.org\/10.1126\/sciadv.adt3177)"},{"key":"bib22","series-title":"Genes Dev","first-page":"1252","article-title":"Max deletion destabilizes MYC protein and abrogates E\u00b5-Myc lymphomagenesis","volume":"33","author":"Mathsyaraja H","year":"2019","unstructured":"Mathsyaraja H\n, \nFreie B\n, \nCheng PF\n, et al.\n Max deletion destabilizes MYC protein and abrogates E\u00b5-Myc lymphomagenesis. Genes Dev 2019 33 1252\u20131264. (https:\/\/doi.org\/10.1101\/gad.325878.119)"},{"key":"bib23","series-title":"Cancer Cell","first-page":"97","article-title":"MAX functions as a tumor suppressor and rewires metabolism in small cell lung cancer","volume":"38","author":"Augert A","year":"2020","unstructured":"Augert A\n, \nMathsyaraja H\n, \nIbrahim AH\n, et al.\n MAX functions as a tumor suppressor and rewires metabolism in small cell lung cancer. Cancer Cell 2020 38 97\u2013114.e7. (https:\/\/doi.org\/10.1016\/j.ccell.2020.04.016)"},{"key":"bib24","series-title":"Mol Cell Biol","first-page":"3470","article-title":"The nerve growth factor-responsive PC12 cell line does not express the Myc dimerization partner Max","volume":"15","author":"Hopewell R","year":"1995","unstructured":"Hopewell R\n & \nZiff EB\n\n. The nerve growth factor-responsive PC12 cell line does not express the Myc dimerization partner Max. Mol Cell Biol 1995 15 3470\u20133478. (https:\/\/doi.org\/10.1128\/mcb.15.7.3470)"},{"key":"bib25","series-title":"Nat Commun","first-page":"2106","article-title":"Epigenetic profiling reveals key genes and cis-regulatory networks specific to human parathyroids","volume":"15","author":"Jung YL","year":"2024","unstructured":"Jung YL\n, \nZhao W\n, \nLi I\n, et al.\n Epigenetic profiling reveals key genes and cis-regulatory networks specific to human parathyroids. Nat Commun 2024 15 2106. (https:\/\/doi.org\/10.1038\/s41467-024-46181-3)"},{"key":"bib26","series-title":"Front Endocrinol","article-title":"Genotype and clinical phenotype characteristics of MAX germline mutation-associated pheochromocytoma\/paraganglioma syndrome","volume":"15","author":"Lian B","year":"2024","unstructured":"Lian B\n, \nLu J\n, \nFang X\n, et al.\n Genotype and clinical phenotype characteristics of MAX germline mutation-associated pheochromocytoma\/paraganglioma syndrome. Front Endocrinol 2024 15 1442691. (https:\/\/doi.org\/10.3389\/fendo.2024.1442691)"},{"key":"bib27","series-title":"Endocr Pract","article-title":"Multiple endocrine neoplasia type 5 due to germline MAX mutations: a systematic review of tumor spectrum and clinical features","author":"Charoenngam N","year":"2025","unstructured":"Charoenngam N\n, \nThaneerat N\n, \nLikasitwatanakul P\n, et al\n. Multiple endocrine neoplasia type 5 due to germline MAX mutations: a systematic review of tumor spectrum and clinical features. Endocr Pract 2025. (https:\/\/doi.org\/10.1016\/j.eprac.2025.12.022)"},{"key":"bib28","series-title":"J Endocr Soc","article-title":"THU613 multiple endocrine neoplasia type 5 associated with germline Max mutations \u2013 new insights into this proposed entity","volume":"7","author":"Charoenngam N","year":"2023","unstructured":"Charoenngam N\n, \nLikasitwatanakul P\n, \nKansuttiviwat C\n, et al.\n THU613 multiple endocrine neoplasia type 5 associated with germline Max mutations \u2013 new insights into this proposed entity. J Endocr Soc 2023 7 (Supplement 1) bvad114.43. (https:\/\/doi.org\/10.1210\/jendso\/bvad114.143)"},{"key":"bib29","series-title":"Anti Cancer Drugs","first-page":"102","article-title":"Temozolomide treatment of a malignant pheochromocytoma and an unresectable MAX-related paraganglioma","volume":"29","author":"Ferrara AM","year":"2018","unstructured":"Ferrara AM\n, \nLombardi G\n, \nPambuku A\n, et al.\n Temozolomide treatment of a malignant pheochromocytoma and an unresectable MAX-related paraganglioma. Anti Cancer Drugs 2018 29 102\u2013105. (https:\/\/doi.org\/10.1097\/cad.0000000000000570)"},{"key":"bib30","series-title":"Front Endocrinol","first-page":"234","article-title":"A novel MAX gene mutation variant in a patient with multiple and \u201ccomposite\u201d neuroendocrine-neuroblastic tumors","volume":"11","author":"Pozza C","year":"2020","unstructured":"Pozza C\n, \nSesti F\n, \nDi Dato C\n, et al.\n A novel MAX gene mutation variant in a patient with multiple and \u201ccomposite\u201d neuroendocrine-neuroblastic tumors. Front Endocrinol 2020 11 234. (https:\/\/doi.org\/10.3389\/fendo.2020.00234)"},{"key":"bib31","series-title":"Endocr Relat Cancer","first-page":"L37","article-title":"Pheochromocytomas and pituitary adenomas in three patients with MAX exon deletions","volume":"25","author":"Daly AF","year":"2018","unstructured":"Daly AF\n, \nCastermans E\n, \nOudijk L\n, et al.\n Pheochromocytomas and pituitary adenomas in three patients with MAX exon deletions. Endocr Relat Cancer 2018 25 L37\u2013L42. (https:\/\/doi.org\/10.1530\/erc-18-0065)"},{"key":"bib32","series-title":"AACE Clin Case Rep","first-page":"e453","article-title":"Case report of bilateral pheochromocytomas due to a novel Max mutation in a patient known to have a pituitary prolactinoma","volume":"4","author":"Kobza AO","year":"2018","unstructured":"Kobza AO\n, \nDizon S\n & \nArnaout A\n\n. Case report of bilateral pheochromocytomas due to a novel Max mutation in a patient known to have a pituitary prolactinoma. AACE Clin Case Rep 2018 4 e453\u2013e456. (https:\/\/doi.org\/10.4158\/accr-2018-0146)"},{"key":"bib33","series-title":"J Endocr Soc","first-page":"1401","article-title":"Case report of a prolactinoma in a patient with a novel MAX mutation and bilateral pheochromocytomas","volume":"1","author":"Roszko KL","year":"2017","unstructured":"Roszko KL\n, \nBlouch E\n, \nBlake M\n, et al.\n Case report of a prolactinoma in a patient with a novel MAX mutation and bilateral pheochromocytomas. J Endocr Soc 2017 1 1401\u20131407. (https:\/\/doi.org\/10.1210\/js.2017-00135)"},{"key":"bib34","series-title":"Front Endocrinol","article-title":"Familial acromegaly and bilateral asynchronous pheochromocytomas in a female patient with a MAX mutation: a case report","volume":"12","author":"Mamedova E","year":"2021","unstructured":"Mamedova E\n, \nVasilyev E\n, \nPetrov V\n, et al.\n Familial acromegaly and bilateral asynchronous pheochromocytomas in a female patient with a MAX mutation: a case report. Front Endocrinol 2021 12 683492. (https:\/\/doi.org\/10.3389\/fendo.2021.683492)"},{"key":"bib35","series-title":"Endocr Relat Cancer","first-page":"R157","article-title":"Succinate dehydrogenase and MYC-associated factor X mutations in pituitary neuroendocrine tumours","volume":"29","author":"Loughrey PB","year":"2022","unstructured":"Loughrey PB\n, \nRoncaroli F\n, \nHealy E\n, et al.\n Succinate dehydrogenase and MYC-associated factor X mutations in pituitary neuroendocrine tumours. Endocr Relat Cancer 2022 29 R157\u2013R172. (https:\/\/doi.org\/10.1530\/erc-22-0157)"},{"key":"bib36","series-title":"JCEM Case Rep","article-title":"Primary hyperparathyroidism in a patient with bilateral pheochromocytoma and a mutation in the tumor suppressor MAX","volume":"1","author":"Charoenngam N","year":"2023","unstructured":"Charoenngam N\n & \nMannstadt M\n\n. Primary hyperparathyroidism in a patient with bilateral pheochromocytoma and a mutation in the tumor suppressor MAX. JCEM Case Rep 2023 1 luad006. (https:\/\/doi.org\/10.1210\/jcemcr\/luad006)"},{"key":"bib37","series-title":"Endocr Pathol","first-page":"64","article-title":"Overview of the 2022 WHO classification of parathyroid tumors","volume":"33","author":"Erickson LA","year":"2022","unstructured":"Erickson LA\n, \nMete O\n, \nJuhlin CC\n, et al.\n Overview of the 2022 WHO classification of parathyroid tumors. Endocr Pathol 2022 33 64\u201389. (https:\/\/doi.org\/10.1007\/s12022-022-09709-1)"},{"key":"bib38","series-title":"J Clin Endocrinol Metab","first-page":"1915","article-title":"Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline","volume":"99","author":"Lenders JWM","year":"2014","unstructured":"Lenders JWM\n, \nDuh Q-Y\n, \nEisenhofer G\n, et al.\n Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014 99 1915\u20131942. (https:\/\/doi.org\/10.1210\/jc.2014-1498)"},{"key":"bib39","series-title":"Clin Endocrinol","first-page":"424","article-title":"Molecular genetic testing in the management of pituitary disease","volume":"97","author":"Coopmans EC","year":"2022","unstructured":"Coopmans EC\n & \nKorbonits M\n\n. Molecular genetic testing in the management of pituitary disease. Clin Endocrinol 2022 97 424\u2013435. (https:\/\/doi.org\/10.1111\/cen.14706)"},{"key":"bib40","series-title":"Eur J Endocrinol","first-page":"369","article-title":"Germinal defects of SDHx genes in patients with isolated pituitary adenoma","volume":"183","author":"Mougel G","year":"2020","unstructured":"Mougel G\n, \nLagarde A\n, \nAlbarel F\n, et al.\n Germinal defects of SDHx genes in patients with isolated pituitary adenoma. Eur J Endocrinol 2020 183 369\u2013379. (https:\/\/doi.org\/10.1530\/eje-20-0054)"},{"key":"bib41","series-title":"JAMA Netw Open","article-title":"Comparison of pheochromocytoma-specific morbidity and mortality among adults with bilateral pheochromocytomas undergoing total adrenalectomy vs cortical-sparing adrenalectomy","volume":"2","author":"Neumann HPH","year":"2019","unstructured":"Neumann HPH\n, \nTsoy U\n, \nBancos I\n, et al.\n Comparison of pheochromocytoma-specific morbidity and mortality among adults with bilateral pheochromocytomas undergoing total adrenalectomy vs cortical-sparing adrenalectomy. JAMA Netw Open 2019 2 e198898. (https:\/\/doi.org\/10.1001\/jamanetworkopen.2019.8898)"},{"key":"bib42","series-title":"Endocr Connect","first-page":"1463","article-title":"Metastatic cluster 2-related pheochromocytoma\/paraganglioma: a single-center experience and systematic review","volume":"10","author":"Kumar S","year":"2021","unstructured":"Kumar S\n, \nLila AR\n, \nMemon SS\n, et al.\n Metastatic cluster 2-related pheochromocytoma\/paraganglioma: a single-center experience and systematic review. Endocr Connect 2021 10 1463\u20131476. (https:\/\/doi.org\/10.1530\/ec-21-0455)"},{"key":"bib43","series-title":"Clin Cancer Res","first-page":"3368","article-title":"Update on tumor surveillance for children with hereditary pheochromocytoma\/paraganglioma syndromes","volume":"31","author":"Rednam SP","year":"2025","unstructured":"Rednam SP\n, \nKamihara J\n, \nBecktell KD\n, et al.\n Update on tumor surveillance for children with hereditary pheochromocytoma\/paraganglioma syndromes. Clin Cancer Res 2025 31 3368\u20133376. (https:\/\/doi.org\/10.1158\/1078-0432.ccr-24-4354)"},{"key":"bib44","series-title":"Version 3.2025.","article-title":"NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines\u00ae): Neuroendocrine and adrenal tumors","author":"National Comprehensive Cancer Network","year":"2025","unstructured":"National Comprehensive Cancer Network\n. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines\u00ae): Neuroendocrine and adrenal tumors. Version 3.2025. Plymouth Meeting, PA, USA: NCCN, 2025. (https:\/\/www.nccn.org\/guidelines\/guidelines-detail?id=1448)"},{"key":"bib45","series-title":"Front Endocrinol","first-page":"558","article-title":"A novel phenotype of germline pathogenic variants in MAX: concurrence of pheochromocytoma and ganglioneuroma in a Chinese family and literature review","volume":"11","author":"Chang X","year":"2020","unstructured":"Chang X\n, \nLi Z\n, \nMa X\n, et al.\n A novel phenotype of germline pathogenic variants in MAX: concurrence of pheochromocytoma and ganglioneuroma in a Chinese family and literature review. Front Endocrinol 2020 11 558. (https:\/\/doi.org\/10.3389\/fendo.2020.00558)"},{"key":"bib1","series-title":"Cancer Res","first-page":"3119","article-title":"MAX and MYC: a heritable breakup","volume":"72","author":"Casc\u00f3n A","year":"2012","unstructured":"Casc\u00f3n A\n & \nRobledo M\n\n. MAX and MYC: a heritable breakup. Cancer Res 2012 72 3119\u20133124. (https:\/\/doi.org\/10.1158\/0008-5472.CAN-11-3891)"},{"key":"bib2","series-title":"Nat Genet","first-page":"663","article-title":"Exome sequencing identifies MAX mutations as a cause of hereditary pheochromocytoma","volume":"43","author":"Comino-M\u00e9ndez I","year":"2011","unstructured":"Comino-M\u00e9ndez I\n, \nGracia-Azn\u00e1rez FJ\n, \nSchiavi F\n, et al.\n Exome sequencing identifies MAX mutations as a cause of hereditary pheochromocytoma. Nat Genet 2011 43 663\u2013667. (https:\/\/doi.org\/10.1038\/ng.861)"},{"key":"bib3","series-title":"Clin Cancer Res","first-page":"2828","article-title":"MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma","volume":"18","author":"Burnichon N","year":"2012","unstructured":"Burnichon N\n, \nCasc\u00f3n A\n, \nSchiavi F\n, et al.\n MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma. Clin Cancer Res 2012 18 2828\u20132837. (https:\/\/doi.org\/10.1158\/1078-0432.ccr-12-0160)"},{"key":"bib4","series-title":"J Clin Endocrinol Metab","first-page":"e1163","article-title":"Multiple endocrine tumors associated with germline MAX mutations: multiple endocrine neoplasia type 5?","volume":"106","author":"Seabrook AJ","year":"2021","unstructured":"Seabrook AJ\n, \nHarris JE\n, \nVelosa SB\n, et al.\n Multiple endocrine tumors associated with germline MAX mutations: multiple endocrine neoplasia type 5? J Clin Endocrinol Metab 2021 106 e1163\u2013e1182. (https:\/\/doi.org\/10.1210\/clinem\/dgaa957)"},{"key":"bib5","series-title":"Pituitary","first-page":"127","article-title":"Loss of heterozygosity and absence of MAX immunostaining in a prolactinoma associated with multiple endocrine neoplasia type 5 (MEN5)","volume":"28","author":"Delemer B","year":"2025","unstructured":"Delemer B\n, \nFlorea SM\n, \nDecoudier B\n, et al.\n Loss of heterozygosity and absence of MAX immunostaining in a prolactinoma associated with multiple endocrine neoplasia type 5 (MEN5). Pituitary 2025 28 127. (https:\/\/doi.org\/10.1007\/s11102-025-01607-z)"},{"key":"bib6","series-title":"Front Endocrinol","article-title":"Case report: pheochromocytoma and synchronous neuroblastoma in a family with hereditary pheochromocytoma associated with a MAX deleterious variant","volume":"12","author":"Duarte DB","year":"2021","unstructured":"Duarte DB\n, \nFerreira L\n, \nSantos AP\n, et al.\n Case report: pheochromocytoma and synchronous neuroblastoma in a family with hereditary pheochromocytoma associated with a MAX deleterious variant. Front Endocrinol 2021 12 609263. (https:\/\/doi.org\/10.3389\/fendo.2021.609263)"},{"key":"bib7","series-title":"J Clin Endocrinol Metab","first-page":"4013","article-title":"Clinical and molecular features of renal and pheochromocytoma\/paraganglioma tumor association syndrome (RAPTAS): case series and literature review","volume":"102","author":"Casey RT","year":"2017","unstructured":"Casey RT\n, \nWarren AY\n, \nMartin JE\n, et al.\n Clinical and molecular features of renal and pheochromocytoma\/paraganglioma tumor association syndrome (RAPTAS): case series and literature review. J Clin Endocrinol Metab 2017 102 4013\u20134022. (https:\/\/doi.org\/10.1210\/jc.2017-00562)"},{"key":"bib8","series-title":"J Clin Endocrinol Metab","first-page":"453","article-title":"Complex MAX rearrangement in a family with malignant pheochromocytoma, renal oncocytoma, and erythrocytosis","volume":"101","author":"Korpershoek E","year":"2016","unstructured":"Korpershoek E\n, \nKoffy D\n, \nEussen BH\n, et al.\n Complex MAX rearrangement in a family with malignant pheochromocytoma, renal oncocytoma, and erythrocytosis. J Clin Endocrinol Metab 2016 101 453\u2013460. (https:\/\/doi.org\/10.1210\/jc.2015-2592)"},{"key":"bib9","series-title":"Horm Metab Res","first-page":"784","article-title":"Pancreatic neuroendocrine neoplasm associated with a familial MAX deletion","volume":"52","author":"Petignot S","year":"2020","unstructured":"Petignot S\n, \nDaly AF\n, \nCastermans E\n, et al.\n Pancreatic neuroendocrine neoplasm associated with a familial MAX deletion. Horm Metab Res 2020 52 784\u2013787. (https:\/\/doi.org\/10.1055\/a-1186-0790)"},{"key":"bib10","series-title":"Endocrine","first-page":"701","article-title":"Expanding the phenotype of multiple endocrine neoplasia type 5 (MEN5): pituitary gigantism, myelolipoma and familial pheochromocytoma due to a germline pathogenic MAX variant","volume":"88","author":"Haider A","year":"2025","unstructured":"Haider A\n, \nSundar J\n, \nBeckers A\n, et al.\n Expanding the phenotype of multiple endocrine neoplasia type 5 (MEN5): pituitary gigantism, myelolipoma and familial pheochromocytoma due to a germline pathogenic MAX variant. Endocrine 2025 88 701\u2013705. (https:\/\/doi.org\/10.1007\/s12020-025-04186-y)"},{"key":"bib11","series-title":"JCEM Case Rep","article-title":"Early-onset sarcoma with germline MAX variant: expanding the spectrum in hereditary pheochromocytoma and paraganglioma","volume":"3","author":"Eren A","year":"2025","unstructured":"Eren A\n, \nBrock PL\n & \nDedhia PH\n\n. Early-onset sarcoma with germline MAX variant: expanding the spectrum in hereditary pheochromocytoma and paraganglioma. JCEM Case Rep 2025 3 luaf119. (https:\/\/doi.org\/10.1210\/jcemcr\/luaf119)"},{"key":"bib12","series-title":"Cell","first-page":"193","article-title":"X-Ray structures of Myc-Max and Mad-Max recognizing DNA: molecular bases of regulation by proto-oncogenic transcription factors","volume":"112","author":"Nair SK","year":"2003","unstructured":"Nair SK\n & \nBurley SK\n\n. X-Ray structures of Myc-Max and Mad-Max recognizing DNA: molecular bases of regulation by proto-oncogenic transcription factors. Cell 2003 112 193\u2013205. (https:\/\/doi.org\/10.1016\/s0092-8674(02)01284-9)"},{"key":"bib13","series-title":"Annu Rev Cell Dev Biol","first-page":"653","article-title":"The Myc\/Max\/Mad network and the transcriptional control of cell behavior","volume":"16","author":"Grandori C","year":"2000","unstructured":"Grandori C\n, \nCowley SM\n, \nJames LP\n, et al.\n The Myc\/Max\/Mad network and the transcriptional control of cell behavior. Annu Rev Cell Dev Biol 2000 16 653\u2013699. (https:\/\/doi.org\/10.1146\/annurev.cellbio.16.1.653)"},{"key":"bib14","series-title":"Genes Dev","first-page":"17","article-title":"Essential role for Max in early embryonic growth and development","volume":"14","author":"Shen-Li H","year":"2000","unstructured":"Shen-Li H\n, \nO\u2019Hagan RC\n, \nHou H Jr\n, et al.\n Essential role for Max in early embryonic growth and development. Genes Dev 2000 14 17\u201322. (https:\/\/doi.org\/10.1101\/gad.14.1.17)"},{"key":"bib15","series-title":"Front Med","first-page":"412","article-title":"The MYC transcription factor network: balancing metabolism, proliferation and oncogenesis","volume":"12","author":"Carroll PA","year":"2018","unstructured":"Carroll PA\n, \nFreie BW\n, \nMathsyaraja H\n, et al.\n The MYC transcription factor network: balancing metabolism, proliferation and oncogenesis. Front Med 2018 12 412\u2013425. (https:\/\/doi.org\/10.1007\/s11684-018-0650-z)"},{"key":"bib16","series-title":"Blood","first-page":"1019","article-title":"Development and survival of MYC-driven lymphomas require the MYC antagonist MNT to curb MYC-induced apoptosis","volume":"135","author":"Nguyen HV","year":"2020","unstructured":"Nguyen HV\n, \nVandenberg CJ\n, \nNg AP\n, et al.\n Development and survival of MYC-driven lymphomas require the MYC antagonist MNT to curb MYC-induced apoptosis. Blood 2020 135 1019\u20131031. (https:\/\/doi.org\/10.1182\/blood.2019003014)"},{"key":"bib17","series-title":"J Biol Chem","first-page":"2001","article-title":"The MNT transcription factor autoregulates its expression and supports proliferation in MYC-associated factor X (MAX)-deficient cells","volume":"295","author":"Lafita-Navarro MC","year":"2020","unstructured":"Lafita-Navarro MC\n, \nLia\u00f1o-Pons J\n, \nQuintanilla A\n, et al.\n The MNT transcription factor autoregulates its expression and supports proliferation in MYC-associated factor X (MAX)-deficient cells. J Biol Chem 2020 295 2001\u20132017. (https:\/\/doi.org\/10.1074\/jbc.ra119.010389)"},{"key":"bib18","series-title":"Cancer Res","first-page":"5565","article-title":"Mnt-deficient mammary glands exhibit impaired involution and tumors with characteristics of Myc overexpression","volume":"66","author":"Toyo-oka K","year":"2006","unstructured":"Toyo-oka K\n, \nBowen TJ\n, \nHirotsune S\n, et al.\n Mnt-deficient mammary glands exhibit impaired involution and tumors with characteristics of Myc overexpression. Cancer Res 2006 66 5565\u20135573. (https:\/\/doi.org\/10.1158\/0008-5472.can-05-2683)"},{"key":"bib19","series-title":"Proc Natl Acad Sci U S A","first-page":"6912","article-title":"Glucose sensing by MondoA:Mlx complexes: a role for hexokinases and direct regulation of thioredoxin-interacting protein expression","volume":"105","author":"Stoltzman CA","year":"2008","unstructured":"Stoltzman CA\n, \nPeterson CW\n, \nBreen KT\n, et al.\n Glucose sensing by MondoA:Mlx complexes: a role for hexokinases and direct regulation of thioredoxin-interacting protein expression. Proc Natl Acad Sci U S A 2008 105 6912\u20136917. (https:\/\/doi.org\/10.1073\/pnas.0712199105)"},{"key":"bib20","series-title":"The Myc\/Max\/Mad Transcription Factor Network","first-page":"255","article-title":"The mlx network: evidence for a parallel Max-like transcriptional network that regulates energy metabolism","author":"Billin AN","year":"2006","unstructured":"Billin AN\n & \nAyer DE\n\n. The mlx network: evidence for a parallel Max-like transcriptional network that regulates energy metabolism. In The Myc\/Max\/Mad Transcription Factor Network, pp 255\u2013278. Ed \n\nRN Eisenman\n\n. Berlin, Heidelberg, Germany: Springer, 2006. (https:\/\/doi.org\/10.1007\/3-540-32952-8_10)"},{"key":"bib21","series-title":"Sci Adv","article-title":"MAX inactivation deregulates the MYC network and induces neuroendocrine neoplasia in multiple tissues","volume":"11","author":"Freie B","year":"2025","unstructured":"Freie B\n, \nIbrahim AH\n, \nCarroll PA\n, et al\n. MAX inactivation deregulates the MYC network and induces neuroendocrine neoplasia in multiple tissues. Sci Adv 2025 11 eadt3177. (https:\/\/doi.org\/10.1126\/sciadv.adt3177)"},{"key":"bib22","series-title":"Genes Dev","first-page":"1252","article-title":"Max deletion destabilizes MYC protein and abrogates E\u00b5-Myc lymphomagenesis","volume":"33","author":"Mathsyaraja H","year":"2019","unstructured":"Mathsyaraja H\n, \nFreie B\n, \nCheng PF\n, et al.\n Max deletion destabilizes MYC protein and abrogates E\u00b5-Myc lymphomagenesis. Genes Dev 2019 33 1252\u20131264. (https:\/\/doi.org\/10.1101\/gad.325878.119)"},{"key":"bib23","series-title":"Cancer Cell","first-page":"97","article-title":"MAX functions as a tumor suppressor and rewires metabolism in small cell lung cancer","volume":"38","author":"Augert A","year":"2020","unstructured":"Augert A\n, \nMathsyaraja H\n, \nIbrahim AH\n, et al.\n MAX functions as a tumor suppressor and rewires metabolism in small cell lung cancer. Cancer Cell 2020 38 97\u2013114.e7. (https:\/\/doi.org\/10.1016\/j.ccell.2020.04.016)"},{"key":"bib24","series-title":"Mol Cell Biol","first-page":"3470","article-title":"The nerve growth factor-responsive PC12 cell line does not express the Myc dimerization partner Max","volume":"15","author":"Hopewell R","year":"1995","unstructured":"Hopewell R\n & \nZiff EB\n\n. The nerve growth factor-responsive PC12 cell line does not express the Myc dimerization partner Max. Mol Cell Biol 1995 15 3470\u20133478. (https:\/\/doi.org\/10.1128\/mcb.15.7.3470)"},{"key":"bib25","series-title":"Nat Commun","first-page":"2106","article-title":"Epigenetic profiling reveals key genes and cis-regulatory networks specific to human parathyroids","volume":"15","author":"Jung YL","year":"2024","unstructured":"Jung YL\n, \nZhao W\n, \nLi I\n, et al.\n Epigenetic profiling reveals key genes and cis-regulatory networks specific to human parathyroids. Nat Commun 2024 15 2106. (https:\/\/doi.org\/10.1038\/s41467-024-46181-3)"},{"key":"bib26","series-title":"Front Endocrinol","article-title":"Genotype and clinical phenotype characteristics of MAX germline mutation-associated pheochromocytoma\/paraganglioma syndrome","volume":"15","author":"Lian B","year":"2024","unstructured":"Lian B\n, \nLu J\n, \nFang X\n, et al.\n Genotype and clinical phenotype characteristics of MAX germline mutation-associated pheochromocytoma\/paraganglioma syndrome. Front Endocrinol 2024 15 1442691. (https:\/\/doi.org\/10.3389\/fendo.2024.1442691)"},{"key":"bib27","series-title":"Endocr Pract","article-title":"Multiple endocrine neoplasia type 5 due to germline MAX mutations: a systematic review of tumor spectrum and clinical features","author":"Charoenngam N","year":"2025","unstructured":"Charoenngam N\n, \nThaneerat N\n, \nLikasitwatanakul P\n, et al\n. Multiple endocrine neoplasia type 5 due to germline MAX mutations: a systematic review of tumor spectrum and clinical features. Endocr Pract 2025. (https:\/\/doi.org\/10.1016\/j.eprac.2025.12.022)"},{"key":"bib28","series-title":"J Endocr Soc","article-title":"THU613 multiple endocrine neoplasia type 5 associated with germline Max mutations \u2013 new insights into this proposed entity","volume":"7","author":"Charoenngam N","year":"2023","unstructured":"Charoenngam N\n, \nLikasitwatanakul P\n, \nKansuttiviwat C\n, et al.\n THU613 multiple endocrine neoplasia type 5 associated with germline Max mutations \u2013 new insights into this proposed entity. J Endocr Soc 2023 7 (Supplement 1) bvad114.43. (https:\/\/doi.org\/10.1210\/jendso\/bvad114.143)"},{"key":"bib29","series-title":"Anti Cancer Drugs","first-page":"102","article-title":"Temozolomide treatment of a malignant pheochromocytoma and an unresectable MAX-related paraganglioma","volume":"29","author":"Ferrara AM","year":"2018","unstructured":"Ferrara AM\n, \nLombardi G\n, \nPambuku A\n, et al.\n Temozolomide treatment of a malignant pheochromocytoma and an unresectable MAX-related paraganglioma. Anti Cancer Drugs 2018 29 102\u2013105. (https:\/\/doi.org\/10.1097\/cad.0000000000000570)"},{"key":"bib30","series-title":"Front Endocrinol","first-page":"234","article-title":"A novel MAX gene mutation variant in a patient with multiple and \u201ccomposite\u201d neuroendocrine-neuroblastic tumors","volume":"11","author":"Pozza C","year":"2020","unstructured":"Pozza C\n, \nSesti F\n, \nDi Dato C\n, et al.\n A novel MAX gene mutation variant in a patient with multiple and \u201ccomposite\u201d neuroendocrine-neuroblastic tumors. Front Endocrinol 2020 11 234. (https:\/\/doi.org\/10.3389\/fendo.2020.00234)"},{"key":"bib31","series-title":"Endocr Relat Cancer","first-page":"L37","article-title":"Pheochromocytomas and pituitary adenomas in three patients with MAX exon deletions","volume":"25","author":"Daly AF","year":"2018","unstructured":"Daly AF\n, \nCastermans E\n, \nOudijk L\n, et al.\n Pheochromocytomas and pituitary adenomas in three patients with MAX exon deletions. Endocr Relat Cancer 2018 25 L37\u2013L42. (https:\/\/doi.org\/10.1530\/erc-18-0065)"},{"key":"bib32","series-title":"AACE Clin Case Rep","first-page":"e453","article-title":"Case report of bilateral pheochromocytomas due to a novel Max mutation in a patient known to have a pituitary prolactinoma","volume":"4","author":"Kobza AO","year":"2018","unstructured":"Kobza AO\n, \nDizon S\n & \nArnaout A\n\n. Case report of bilateral pheochromocytomas due to a novel Max mutation in a patient known to have a pituitary prolactinoma. AACE Clin Case Rep 2018 4 e453\u2013e456. (https:\/\/doi.org\/10.4158\/accr-2018-0146)"},{"key":"bib33","series-title":"J Endocr Soc","first-page":"1401","article-title":"Case report of a prolactinoma in a patient with a novel MAX mutation and bilateral pheochromocytomas","volume":"1","author":"Roszko KL","year":"2017","unstructured":"Roszko KL\n, \nBlouch E\n, \nBlake M\n, et al.\n Case report of a prolactinoma in a patient with a novel MAX mutation and bilateral pheochromocytomas. J Endocr Soc 2017 1 1401\u20131407. (https:\/\/doi.org\/10.1210\/js.2017-00135)"},{"key":"bib34","series-title":"Front Endocrinol","article-title":"Familial acromegaly and bilateral asynchronous pheochromocytomas in a female patient with a MAX mutation: a case report","volume":"12","author":"Mamedova E","year":"2021","unstructured":"Mamedova E\n, \nVasilyev E\n, \nPetrov V\n, et al.\n Familial acromegaly and bilateral asynchronous pheochromocytomas in a female patient with a MAX mutation: a case report. Front Endocrinol 2021 12 683492. (https:\/\/doi.org\/10.3389\/fendo.2021.683492)"},{"key":"bib35","series-title":"Endocr Relat Cancer","first-page":"R157","article-title":"Succinate dehydrogenase and MYC-associated factor X mutations in pituitary neuroendocrine tumours","volume":"29","author":"Loughrey PB","year":"2022","unstructured":"Loughrey PB\n, \nRoncaroli F\n, \nHealy E\n, et al.\n Succinate dehydrogenase and MYC-associated factor X mutations in pituitary neuroendocrine tumours. Endocr Relat Cancer 2022 29 R157\u2013R172. (https:\/\/doi.org\/10.1530\/erc-22-0157)"},{"key":"bib36","series-title":"JCEM Case Rep","article-title":"Primary hyperparathyroidism in a patient with bilateral pheochromocytoma and a mutation in the tumor suppressor MAX","volume":"1","author":"Charoenngam N","year":"2023","unstructured":"Charoenngam N\n & \nMannstadt M\n\n. Primary hyperparathyroidism in a patient with bilateral pheochromocytoma and a mutation in the tumor suppressor MAX. JCEM Case Rep 2023 1 luad006. (https:\/\/doi.org\/10.1210\/jcemcr\/luad006)"},{"key":"bib37","series-title":"Endocr Pathol","first-page":"64","article-title":"Overview of the 2022 WHO classification of parathyroid tumors","volume":"33","author":"Erickson LA","year":"2022","unstructured":"Erickson LA\n, \nMete O\n, \nJuhlin CC\n, et al.\n Overview of the 2022 WHO classification of parathyroid tumors. Endocr Pathol 2022 33 64\u201389. (https:\/\/doi.org\/10.1007\/s12022-022-09709-1)"},{"key":"bib38","series-title":"J Clin Endocrinol Metab","first-page":"1915","article-title":"Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline","volume":"99","author":"Lenders JWM","year":"2014","unstructured":"Lenders JWM\n, \nDuh Q-Y\n, \nEisenhofer G\n, et al.\n Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014 99 1915\u20131942. (https:\/\/doi.org\/10.1210\/jc.2014-1498)"},{"key":"bib39","series-title":"Clin Endocrinol","first-page":"424","article-title":"Molecular genetic testing in the management of pituitary disease","volume":"97","author":"Coopmans EC","year":"2022","unstructured":"Coopmans EC\n & \nKorbonits M\n\n. Molecular genetic testing in the management of pituitary disease. Clin Endocrinol 2022 97 424\u2013435. (https:\/\/doi.org\/10.1111\/cen.14706)"},{"key":"bib40","series-title":"Eur J Endocrinol","first-page":"369","article-title":"Germinal defects of SDHx genes in patients with isolated pituitary adenoma","volume":"183","author":"Mougel G","year":"2020","unstructured":"Mougel G\n, \nLagarde A\n, \nAlbarel F\n, et al.\n Germinal defects of SDHx genes in patients with isolated pituitary adenoma. Eur J Endocrinol 2020 183 369\u2013379. (https:\/\/doi.org\/10.1530\/eje-20-0054)"},{"key":"bib41","series-title":"JAMA Netw Open","article-title":"Comparison of pheochromocytoma-specific morbidity and mortality among adults with bilateral pheochromocytomas undergoing total adrenalectomy vs cortical-sparing adrenalectomy","volume":"2","author":"Neumann HPH","year":"2019","unstructured":"Neumann HPH\n, \nTsoy U\n, \nBancos I\n, et al.\n Comparison of pheochromocytoma-specific morbidity and mortality among adults with bilateral pheochromocytomas undergoing total adrenalectomy vs cortical-sparing adrenalectomy. JAMA Netw Open 2019 2 e198898. (https:\/\/doi.org\/10.1001\/jamanetworkopen.2019.8898)"},{"key":"bib42","series-title":"Endocr Connect","first-page":"1463","article-title":"Metastatic cluster 2-related pheochromocytoma\/paraganglioma: a single-center experience and systematic review","volume":"10","author":"Kumar S","year":"2021","unstructured":"Kumar S\n, \nLila AR\n, \nMemon SS\n, et al.\n Metastatic cluster 2-related pheochromocytoma\/paraganglioma: a single-center experience and systematic review. Endocr Connect 2021 10 1463\u20131476. (https:\/\/doi.org\/10.1530\/ec-21-0455)"},{"key":"bib43","series-title":"Clin Cancer Res","first-page":"3368","article-title":"Update on tumor surveillance for children with hereditary pheochromocytoma\/paraganglioma syndromes","volume":"31","author":"Rednam SP","year":"2025","unstructured":"Rednam SP\n, \nKamihara J\n, \nBecktell KD\n, et al.\n Update on tumor surveillance for children with hereditary pheochromocytoma\/paraganglioma syndromes. Clin Cancer Res 2025 31 3368\u20133376. (https:\/\/doi.org\/10.1158\/1078-0432.ccr-24-4354)"},{"key":"bib44","series-title":"Version 3.2025.","article-title":"NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines\u00ae): Neuroendocrine and adrenal tumors","author":"National Comprehensive Cancer Network","year":"2025","unstructured":"National Comprehensive Cancer Network\n. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines\u00ae): Neuroendocrine and adrenal tumors. Version 3.2025. Plymouth Meeting, PA, USA: NCCN, 2025. (https:\/\/www.nccn.org\/guidelines\/guidelines-detail?id=1448)"},{"key":"bib45","series-title":"Front Endocrinol","first-page":"558","article-title":"A novel phenotype of germline pathogenic variants in MAX: concurrence of pheochromocytoma and ganglioneuroma in a Chinese family and literature review","volume":"11","author":"Chang X","year":"2020","unstructured":"Chang X\n, \nLi Z\n, \nMa X\n, et al.\n A novel phenotype of germline pathogenic variants in MAX: concurrence of pheochromocytoma and ganglioneuroma in a Chinese family and literature review. Front Endocrinol 2020 11 558. 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