{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T23:15:54Z","timestamp":1793661354461,"version":"4.5.1"},"reference-count":31,"publisher":"American Society of Clinical Oncology (ASCO)","issue":"28","content-domain":{"domain":["ascopubs.org"],"crossmark-restriction":true},"short-container-title":["J Clin Oncol"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:sec>\n                    <jats:title>PURPOSE<\/jats:title>\n                    <jats:p>\n                      <jats:sup>177<\/jats:sup>\n                      Lu-DOTA(0)-Tyr(3)-octreotate (\n                      <jats:sup>177<\/jats:sup>\n                      Lu-DOTATATE) is a somatostatin receptor (SSTR)-targeting radiopharmaceutical that shows promise for treating metastatic pheochromocytomas\/paragangliomas (PPGLs), a rare SSTR-expressing tumor.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>\n                      In the first stage of this two-stage Simon phase II trial, 36 PPGL patients with RECIST 1.1 progression within 12 months were prospectively recruited into two genetic cohorts (succinate dehydrogenase [\n                      <jats:italic toggle=\"yes\">SDHx<\/jats:italic>\n                      ]\u2013mutated\n                      <jats:italic toggle=\"yes\">v<\/jats:italic>\n                      apparent sporadic, 18 per cohort) and treated with four cycles of\n                      <jats:sup>177<\/jats:sup>\n                      Lu-DOTATATE. The primary end point was progression-free survival (PFS) rate at 6 months (from initiation of treatment). Secondary end points included safety, overall survival (OS), response rate, imaging\/serum biomarkers, and antihypertensive medication reduction. Computed tomography\/magnetic resonance imaging (CT\/MRIs) and positron emission tomography (PET)-CTs (\n                      <jats:sup>68<\/jats:sup>\n                      Ga-DOTATATE and\n                      <jats:sup>18<\/jats:sup>\n                      F-labeled fluorodeoxyglucose) were obtained after two and four cycles, then every 3 (CT\/MRIs) to 6 months (PET\/CTs). Patients with systolic blood pressure (SBP) &gt; 200 mmHg despite medical management were treated in the intensive care unit (ICU).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>\n                      Six-month PFS rate for all patients was 0.861 (95% CI, 0.755 to 0.982), which was significantly lower (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      = .009) for\n                      <jats:italic toggle=\"yes\">SDHx<\/jats:italic>\n                      at 0.72 (95% CI, 0.542 to 0.962) versus sporadic at 1.00 (95% CI, 1.0 to 1.0). Median PFS was 19.9 months (12.9 months\n                      <jats:italic toggle=\"yes\">SDHx v<\/jats:italic>\n                      24.3 months sporadic) and median OS was 51.7 months (31.2 months\n                      <jats:italic toggle=\"yes\">SDHx v<\/jats:italic>\n                      not reached in sporadic). Best response was achieved on average 11.0 months after completing\n                      <jats:sup>177<\/jats:sup>\n                      Lu-DOTATATE. A 17% incidence of grade 3+ catecholamine release syndrome (CRS) was noted, which may benefit from preemptive ICU admission. Plasma chromogranin A and normetanephrine were the best tumor-marker surrogates and correlated well with changes in RECIST sum and total tumor lesion uptake on serial\n                      <jats:sup>68<\/jats:sup>\n                      Ga-DOTATATE PET-CT scans.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSION<\/jats:title>\n                    <jats:p>\n                      <jats:sup>177<\/jats:sup>\n                      Lu-DOTATATE demonstrated effectiveness and acceptable safety profile for progressive, metastatic PPGL. CRS may occur but can be mitigated through pretreatment with antihypertensives, and, when appropriate, intensified monitoring in the ICU with intravenous antihypertensives.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1200\/jco-25-00791","type":"journal-article","created":{"date-parts":[[2025,8,19]],"date-time":"2025-08-19T19:59:09Z","timestamp":1755633549000},"page":"3102-3112","update-policy":"https:\/\/doi.org\/10.1200\/crossmark","source":"Crossref","is-referenced-by-count":25,"title":["Phase II Study of\n                    <sup>177<\/sup>\n                    Lu-DOTATATE for Progressive Metastatic Pheochromocytomas and Paragangliomas: Interim Analysis of Efficacy, Safety, and Biomarkers"],"prefix":"10.1200","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3539-1454","authenticated-orcid":true,"given":"Frank I.","family":"Lin","sequence":"first","affiliation":[{"name":"Molecular Imaging Branch, National Institutes of Health, National Cancer Institute, Center for Cancer Research, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9710-4030","authenticated-orcid":true,"given":"Jaydira","family":"del Rivero","sequence":"additional","affiliation":[{"name":"Developmental Therapeutics Branch, National Institutes of Health, National Cancer Institute, Center for Cancer Research, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8513-5734","authenticated-orcid":true,"given":"Jorge A.","family":"Carrasquillo","sequence":"additional","affiliation":[{"name":"Molecular Imaging Branch, National Institutes of Health, National Cancer Institute, Center for Cancer Research, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1489-2471","authenticated-orcid":true,"given":"Abhishek","family":"Jha","sequence":"additional","affiliation":[{"name":"National Institutes of Health, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joy","family":"Zou","sequence":"additional","affiliation":[{"name":"Molecular Imaging Branch, National Institutes of Health, National Cancer Institute, Center for Cancer Research, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inna","family":"Shamis","sequence":"additional","affiliation":[{"name":"Molecular Imaging Branch, National Institutes of Health, National Cancer Institute, Center for Cancer Research, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-6969-4735","authenticated-orcid":true,"given":"Sara","family":"Talvacchio","sequence":"additional","affiliation":[{"name":"National Institutes of Health, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0853-6494","authenticated-orcid":false,"given":"Baris","family":"Turkbey","sequence":"additional","affiliation":[{"name":"Molecular Imaging Branch, National Institutes of Health, National Cancer Institute, Center for Cancer Research, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erich P.","family":"Huang","sequence":"additional","affiliation":[{"name":"Division of Cancer Therapy and Diagnosis, Biometric Research Program, National Institutes of Health, National Cancer Institute, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanna","family":"Shih","sequence":"additional","affiliation":[{"name":"Division of Cancer Therapy and Diagnosis, Biometric Research Program, National Institutes of Health, National Cancer Institute, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanna","family":"Klubo-Gwiezdzinska","sequence":"additional","affiliation":[{"name":"Metabolic Diseases Branch, National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esther","family":"Mena","sequence":"additional","affiliation":[{"name":"Molecular Imaging Branch, National Institutes of Health, National Cancer Institute, Center for Cancer Research, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8514-0648","authenticated-orcid":true,"given":"Liza","family":"Lindenberg","sequence":"additional","affiliation":[{"name":"Molecular Imaging Branch, National Institutes of Health, National Cancer Institute, Center for Cancer Research, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6568-9783","authenticated-orcid":true,"given":"Yating","family":"Teng","sequence":"additional","affiliation":[{"name":"Uniformed Services University of the Health Sciences, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0727-3242","authenticated-orcid":true,"given":"Freddy E.","family":"Escorcia","sequence":"additional","affiliation":[{"name":"Molecular Imaging Branch, National Institutes of Health, National Cancer Institute, Center for Cancer Research, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clara","family":"Chen","sequence":"additional","affiliation":[{"name":"Division of Nuclear Medicine, National Institutes of Health, Clinical Center, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Herscovitch","sequence":"additional","affiliation":[{"name":"PET Department, National Institutes of Health, Clinical Center, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corina","family":"Millo","sequence":"additional","affiliation":[{"name":"PET Department, National Institutes of Health, Clinical Center, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter L.","family":"Choyke","sequence":"additional","affiliation":[{"name":"Molecular Imaging Branch, National Institutes of Health, National Cancer Institute, Center for Cancer Research, Bethesda, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3541-3767","authenticated-orcid":true,"given":"Karel","family":"Pacak","sequence":"additional","affiliation":[{"name":"National Institutes of Health, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD"},{"name":"AKESO, Center for Adrenal Endocrine Tumors, Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"233","reference":[{"key":"e_1_3_9_2_2","doi-asserted-by":"publisher","DOI":"10.1007\/s12022-022-09704-6"},{"key":"e_1_3_9_3_2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2017-00992"},{"key":"e_1_3_9_4_2","doi-asserted-by":"publisher","DOI":"10.1210\/endrev\/bnab019"},{"key":"e_1_3_9_5_2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2018-01968"},{"key":"e_1_3_9_6_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00259-016-3357-x"},{"key":"e_1_3_9_7_2","doi-asserted-by":"publisher","DOI":"10.2967\/jnumed.118.211706"},{"key":"e_1_3_9_8_2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2019-00018"},{"key":"e_1_3_9_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cpet.2015.06.001"},{"key":"e_1_3_9_10_2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2017-00816"},{"key":"e_1_3_9_11_2","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-14-2751"},{"key":"e_1_3_9_12_2","volume-title":"NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines\u00ae) for Neuroendocrine and Adrenal Tumors V2","author":"NCCN","year":"2024","unstructured":"NCCN: NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines\u00ae) for Neuroendocrine and Adrenal Tumors V2. 2024. https:\/\/www.nccn.org"},{"key":"e_1_3_9_13_2","doi-asserted-by":"publisher","DOI":"10.1097\/MPA.0000000000001792"},{"key":"e_1_3_9_14_2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2013-1907"},{"key":"e_1_3_9_15_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41574-023-00926-0"},{"key":"e_1_3_9_16_2","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2023.1279828"},{"key":"e_1_3_9_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(23)02554-0"},{"key":"e_1_3_9_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(24)00133-5"},{"key":"e_1_3_9_19_2","doi-asserted-by":"publisher","DOI":"10.1210\/jcemcr\/luae049"},{"key":"e_1_3_9_20_2","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2023.1275813"},{"key":"e_1_3_9_21_2","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2022.957172"},{"key":"e_1_3_9_22_2","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-18-0901"},{"key":"e_1_3_9_23_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.esmoop.2021.100171"},{"key":"e_1_3_9_24_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1464-410X.2008.07558.x"},{"key":"e_1_3_9_25_2","volume-title":"FDA Label for Lutathera","year":"2024","unstructured":"FDA Label for Lutathera. 2024. https:\/\/www.accessdata.fda.gov\/drugsatfda_docs\/label\/2024\/208700s031lbl.pdf"},{"key":"e_1_3_9_26_2","doi-asserted-by":"publisher","DOI":"10.1159\/000507761"},{"key":"e_1_3_9_27_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00259-009-1151-8"},{"key":"e_1_3_9_28_2","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-16-2743"},{"key":"e_1_3_9_29_2","article-title":"Belzutifan monotherapy in Chinese patients (pts) with von Hippel-Lindau (VHL) disease\u2013associated tumors: Results of LITESPARK-015 study","volume":"43","author":"Qui Jz J","year":"2025","unstructured":"Qui Jz J, Cai L, Kong W, et al: Belzutifan monotherapy in Chinese patients (pts) with von Hippel-Lindau (VHL) disease\u2013associated tumors: Results of LITESPARK-015 study. J Clin Oncol 43, 2025 (suppl 5; abstr 534)","journal-title":"J Clin Oncol"},{"key":"e_1_3_9_30_2","unstructured":"FDA: FDA approves belzutifan for pheochromocytoma or paraganglioma. 2025. https:\/\/www.fda.gov\/drugs\/resources-information-approved-drugs\/fda-approves-belzutifan-pheochromocytoma-or-paraganglioma"},{"key":"e_1_3_9_31_2","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgae252"},{"key":"e_1_3_9_32_2","doi-asserted-by":"publisher","DOI":"10.1530\/ERC-22-0236"}],"updated-by":[{"DOI":"10.1200\/jco-25-02180","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2025,9,26]],"date-time":"2025-09-26T00:00:00Z","timestamp":1758844800000}}],"container-title":["Journal of Clinical Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/ascopubs.org\/doi\/pdfdirect\/10.1200\/JCO-25-00791","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,26]],"date-time":"2025-09-26T21:59:15Z","timestamp":1758923955000},"score":1,"resource":{"primary":{"URL":"https:\/\/ascopubs.org\/doi\/10.1200\/JCO-25-00791"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10]]},"references-count":31,"journal-issue":{"issue":"28","published-print":{"date-parts":[[2025,10]]}},"alternative-id":["10.1200\/JCO-25-00791"],"URL":"https:\/\/doi.org\/10.1200\/jco-25-00791","relation":{"is-referenced-by":[{"id-type":"doi","id":"10.1007\/s40137-026-00509-8","asserted-by":"object"}]},"ISSN":["0732-183X","1527-7755"],"issn-type":[{"value":"0732-183X","type":"print"},{"value":"1527-7755","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10]]},"assertion":[{"value":"2025-04-08","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-07-08","order":1,"name":"revised","label":"Revised","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-07-21","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-08-19","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}