{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T16:29:19Z","timestamp":1787070559222,"version":"build-2736575974"},"reference-count":142,"publisher":"The Endocrine Society","license":[{"start":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T00:00:00Z","timestamp":1786320000000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine neoplasms arising from the sympathetic and parasympathetic nervous systems. Their heterogeneous clinical behavior and metastatic potential present significant management challenges. Pseudohypoxia driven by oxygen metabolism dysregulation is the central oncogenic mechanism in cluster 1 PPGLs, which carry the highest metastatic risk. In these tumors, pathogenic variants in the succinate dehydrogenase complex (SDHx) or von Hippel\u2013Lindau (VHL) gene create an intracellular environment that mimics chronic hypoxia, leading to hypoxia-inducible factor 2 alpha (HIF 2\u03b1) activation and driving the hypervascularity and aggressive clinical phenotype. Belzutifan, a first in class selective HIF 2\u03b1 inhibitor, has emerged as a promising therapeutic strategy for pseudohypoxia driven PPGLs. In the LITESPARK 015 phase 2 trial, belzutifan achieved a disease control rate of 85% and an overall response rate of 26% in patients with locally advanced or metastatic disease. Beyond radiographic outcomes, belzutifan provides meaningful clinical benefits, including improved blood pressure control and enhanced quality of life. Unlike cytotoxic therapies, belzutifan's safety profile is characterized by predictable, mechanism-based adverse events, most commonly grade 1 to 3 anemia. Belzutifan is the first oral and the second Food and Drug Administration\u2013approved therapy for metastatic PPGLs establishing a new therapeutic option for these complex rare tumors. While its development underscores the utility of precision oncology, definitive molecular correlations with clinical efficacy remain to be demonstrated.<\/jats:p>","DOI":"10.1210\/clinem\/dgag324","type":"journal-article","created":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T11:17:05Z","timestamp":1786101425000},"source":"Crossref","is-referenced-by-count":0,"title":["Belzutifan, a hypoxia-inducible factor 2-alpha inhibitor, for patients with advanced pheochromocytoma and paraganglioma"],"prefix":"10.1210","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5292-3355","authenticated-orcid":false,"given":"Camilo","family":"Jimenez","sequence":"first","affiliation":[{"name":"Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center , Houston, TX 77030 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0280-0100","authenticated-orcid":false,"given":"Steven G","family":"Waguespack","sequence":"additional","affiliation":[{"name":"Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center , Houston, TX 77030 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Kaplan","sequence":"additional","affiliation":[{"name":"Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center , Houston, TX 77030 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mouhammed Amir","family":"Habra","sequence":"additional","affiliation":[{"name":"Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center , Houston, TX 77030 ,","place":["USA"]},{"name":"Section of Endocrinology, King Faisal Specialist Hospital and Research Centre , Riyadh 11211 ,","place":["Saudi Arabia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeena","family":"Varghese","sequence":"additional","affiliation":[{"name":"Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center , Houston, TX 77030 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2026,8,10]]},"reference":[{"issue":"1","key":"2026081811271139200_dgag324-B1","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1007\/s12022-022-09704-6","article-title":"Overview of the 2022 WHO classification of paragangliomas and pheochromocytomas","volume":"33","author":"Mete","year":"2022","journal-title":"Endocr Pathol"},{"issue":"3","key":"2026081811271139200_dgag324-B2","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1530\/EJE-15-0456","article-title":"Constipation: an overlooked, unmanaged symptom of patients with pheochromocytoma and sympathetic 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