{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T20:04:27Z","timestamp":1792785867895,"version":"4.5.0"},"reference-count":59,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2023,11,9]],"date-time":"2023-11-09T00:00:00Z","timestamp":1699488000000},"content-version":"vor","delay-in-days":8,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"German Research Foundation","doi-asserted-by":"publisher","award":["314061271 \u2013 TRR 205"],"award-info":[{"award-number":["314061271 \u2013 TRR 205"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"University Medicine Zurich"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,11,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>The therapeutic options for metastatic pheochromocytomas\/paragangliomas (mPPGLs) include chemotherapy with cyclophosphamide\/vincristine\/dacarbazine (CVD), temozolomide monotherapy, radionuclide therapies, and tyrosine kinase inhibitors such as sunitinib. The objective of this multicenter retrospective study was to evaluate and compare the responses of mPPGLs including those with pathogenic variants in succinate dehydrogenase subunit B (SDHB), to different systemic treatments.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Design<\/jats:title>\n                    <jats:p>This is a retrospective analysis of treatment responses of mPPGL patients (n = 74) to systemic therapies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients with mPPGLs treated at 6 specialized national centers were selected based on participation in the ENSAT registry. Survival until detected progression (SDP) and disease-control rates (DCRs) at 3 months were evaluated based on imaging reports.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>For the group of patients with progressive disease at baseline (83.8% of 74 patients), the DCR with first-line CVD chemotherapy was 75.0% (n = 4, SDP 11 months; SDHB [n = 1]: DCR 100%, SDP 30 months), with somatostatin peptide receptor-based radionuclide therapy (PPRT) 85.7% (n = 21, SDP 17 months; SDHB [n = 10]: DCR 100%, SDP 14 months), with 131I-meta-iodobenzylguanidine (131I-MIBG) 82.6% (n = 23, SDP 43 months; SDHB [n = 4]: DCR 100%, SDP 24 months), with sunitinib 100% (n = 7, SDP 18 months; SDHB [n = 3]: DCR 100%, SDP 18 months), and with somatostatin analogs 100% (n = 4, SDP not reached). The DCR with temozolomide as second-line therapy was 60.0% (n = 5, SDP 10 months; SDHB [n = 4]: DCR 75%, SDP 10 months).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>We demonstrate in a real-life clinical setting that all current therapies show reasonable efficacy in preventing disease progression, and this is equally true for patients with germline SDHB mutations.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ejendo\/lvad146","type":"journal-article","created":{"date-parts":[[2023,11,10]],"date-time":"2023-11-10T20:46:50Z","timestamp":1699649210000},"page":"546-565","source":"Crossref","is-referenced-by-count":23,"title":["Responses to systemic therapy in metastatic pheochromocytoma\/paraganglioma: a retrospective multicenter cohort study"],"prefix":"10.1093","volume":"189","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4555-8334","authenticated-orcid":false,"given":"Alessa","family":"Fischer","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH) , Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Kloos","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH) , Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3499-5910","authenticated-orcid":false,"given":"Hanna","family":"Remde","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of W\u00fcrzburg , W\u00fcrzburg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich","family":"Dischinger","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of W\u00fcrzburg , W\u00fcrzburg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Pamporaki","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine III, University Hospital Carl Gustav Carus, Technische Universit\u00e4t Dresden , Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henri J L M","family":"Timmers","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Department of Internal Medicine, Radboud University Medical Center , Nijmegen , Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mercedes","family":"Robledo","sequence":"additional","affiliation":[{"name":"Hereditary Endocrine Cancer Group, Spanish National Cancer Research Center (CNIO) , Madrid , Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras , Institute de Salud Carlos III, Madrid , Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5515-8196","authenticated-orcid":false,"given":"Stephanie M J","family":"Fliedner","sequence":"additional","affiliation":[{"name":"First Department of Medicine, University Medical Center Schleswig-Holstein , L\u00fcbeck , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7795-1395","authenticated-orcid":false,"given":"Katharina","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian","family":"Maurer","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Astrid","family":"Reul","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH) , Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6932-333X","authenticated-orcid":false,"given":"Nicole","family":"Bechmann","sequence":"additional","affiliation":[{"name":"Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universita\u0308t Dresden, Fetscherstrasse , Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constanze","family":"Hantel","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH) , Zurich , Switzerland"},{"name":"Department of Internal Medicine III, University Hospital Carl Gustav Carus, Technische Universit\u00e4t Dresden , Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hermine","family":"Mohr","sequence":"additional","affiliation":[{"name":"Institute for Diabetes and Cancer, Helmholtz Zentrum M\u00fcnchen , Neuherberg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia S","family":"Pellegata","sequence":"additional","affiliation":[{"name":"Institute for Diabetes and Cancer, Helmholtz Zentrum M\u00fcnchen , Neuherberg , Germany"},{"name":"Department of Biology and Biotechnology, University of Pavia , Pavia , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan R","family":"Bornstein","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH) , Zurich , Switzerland"},{"name":"Department of Internal Medicine III, University Hospital Carl Gustav Carus, Technische Universit\u00e4t Dresden , Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Kroiss","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph J","family":"Auernhammer","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , Munich , 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