{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T12:30:11Z","timestamp":1777984211406,"version":"3.51.4"},"reference-count":61,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,4,3]],"date-time":"2026-04-03T00:00:00Z","timestamp":1775174400000},"content-version":"vor","delay-in-days":2,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Neuroendocrinology"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Metastatic pheochromocytomas and paragangliomas (mPPGLs) are uncommon, heterogeneous neuroendocrine tumors lacking standardized systemic treatment pathways. Evidence on treatment response predictors and outcome\u2010based stratification remains limited. We conducted a retrospective study of 49 patients with mPPGLs treated between 2010 and 2024 at two Spanish referral centers. We evaluated clinical characteristics, systemic treatment patterns, radiologic responses (per RECIST), and survival outcomes. Patients were stratified into five clinical evolution patterns based on treatment response and disease trajectory. The most common indications for initiating systemic therapy were radiologic progression (59.5%) and high tumor burden (31%). First\u2010line treatments included somatostatin analogues (SSAs, 40.5%), radionuclide therapies (33.3%:\n                    <jats:sup>177<\/jats:sup>\n                    Lu 9.5% and\n                    <jats:sup>131<\/jats:sup>\n                    I\u2010MIBG 23.8%), and chemotherapy (23.8%). Partial response rates were higher with chemotherapy,\n                    <jats:sup>131<\/jats:sup>\n                    I\u2010MIBG, and\n                    <jats:sup>177<\/jats:sup>\n                    Lu compared with SSAs. Tumor burden at treatment initiation appeared to be more closely associated with radiologic response than radiologic progression. Progression\u2010free survival (PFS) appeared to differ according to first\u2010line treatment type, with longer PFS observed in patients receiving radionuclide therapies. The median overall survival from systemic treatment initiation was 48\u2009months. Five clinical evolution patterns were identified, highlighting disease heterogeneity. Radiologic progression remains the main trigger for systemic treatment in mPPGLs; however, initial tumor burden appears to be a stronger predictor of treatment response. Our proposed five\u2010pattern clinical classification may contribute to prognostication and therapeutic individualization. Prospective studies are a key unmet need to determine the optimal timing and sequencing of systemic therapies in mPPGL.\n                  <\/jats:p>","DOI":"10.1111\/jne.70175","type":"journal-article","created":{"date-parts":[[2026,4,4]],"date-time":"2026-04-04T03:25:08Z","timestamp":1775273108000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Patterns of response and survival in metastatic paragangliomas and pheochromocytomas: Experience from two Spanish neuroendocrine tumor centers"],"prefix":"10.1111","volume":"38","author":[{"given":"M. I.","family":"del Olmo\u2010Garc\u00eda","sequence":"first","affiliation":[{"name":"Department of Endocrinology and Nutrition University and Polytechnic Hospital La Fe  Valencia Spain"},{"name":"Joint Research Unit on Endocrinology, Nutrition and Clinical Dietetics University of Valencia\u2010Health Research Institute La Fe  Valencia Spain"},{"name":"Department of Medicine, Faculty of Medicine University of Valencia  Valencia Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. E.","family":"Contreras Saldarriaga","sequence":"additional","affiliation":[{"name":"Universidad de Antioquia Departamento de Endocrinolog\u00eda, Facultad de Medicina, La Candelaria  Medell\u00edn Antioquia Colombia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Hernandez\u2010Rienda","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Nutrition University and Polytechnic Hospital La Fe  Valencia Spain"},{"name":"Joint Research Unit on Endocrinology, Nutrition and Clinical Dietetics University of Valencia\u2010Health Research Institute La Fe  Valencia Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Hernando\u2010Cubero","sequence":"additional","affiliation":[{"name":"Department of Oncology Hospital Universitario Vall Hebron y VHIO\u2014Vall Hebron Instituto de Oncologia  Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Garc\u00eda Alvarez","sequence":"additional","affiliation":[{"name":"Department of Oncology Hospital Universitario Vall Hebron y VHIO\u2014Vall Hebron Instituto de Oncologia  Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7160-8908","authenticated-orcid":false,"given":"A.","family":"Garc\u00eda\u2010Burillo","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine Hospital Universitario Vall Hebron y VHIO\u2014Vall Hebron Instituto de Oncologia  Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Caster\u00e1s","sequence":"additional","affiliation":[{"name":"Department of Endocrinology Hospital Universitario Vall Hebron y VHIO\u2014Vall Hebron Instituto de Oncologia  Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Segura\u2010Huerta","sequence":"additional","affiliation":[{"name":"Department of Oncology University and Polytechnic Hospital La Fe  Valencia Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Prado","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine University and Polytechnic Hospital La Fe  Valencia Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Palas\u00ed","sequence":"additional","affiliation":[{"name":"Department of Surgery University and Polytechnic Hospital La Fe  Valencia Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. 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