{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:46:21Z","timestamp":1791297981953,"version":"4.1.0"},"reference-count":35,"publisher":"The Endocrine Society","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012,11,1]]},"abstract":"\n Context:<\/jats:title>\n Patients with progressive metastatic pheochromocytomas (PHEOs) or sympathetic paragangliomas (SPGLs) face a dismal prognosis. Current systemic therapies are limited.<\/jats:p>\n <\/jats:sec>\n \n Objectives:<\/jats:title>\n The primary end point was progression-free survival determined by RECIST 1.1 criteria or positron emission tomography with [18F]fluorodeoxyglucose\/computed tomography ([18F]FDG-PET\/CT), in the absence of measurable soft tissue targets. Secondary endpoints were tumor response according to RECIST criteria version 1.1 or FDG uptake, blood pressure control, and safety.<\/jats:p>\n <\/jats:sec>\n \n Design:<\/jats:title>\n We conducted a retrospective review of medical records of patients with metastatic PHEO\/SPGL treated with sunitinib from December 2007 through December 2011. An intention-to-treat analysis was performed.<\/jats:p>\n <\/jats:sec>\n \n Patients and Setting:<\/jats:title>\n Seventeen patients with progressive metastatic PHEO\/SPGLs treated at the Institut Gustave-Roussy and MD Anderson Cancer Center.<\/jats:p>\n <\/jats:sec>\n \n Interventions:<\/jats:title>\n Patients treated with sunitinib.<\/jats:p>\n <\/jats:sec>\n \n Results:<\/jats:title>\n According to RECIST 1.1, eight patients experienced clinical benefit; three experienced partial response, and five had stable disease, including four with predominant skeletal metastases that showed a 30% or greater reduction in glucose uptake on [18F]FDG-PET\/CT. Of 14 patients who had hypertension, six became normotensive and two discontinued antihypertensives. One patient treated with sunitinib and rapamycin experienced a durable benefit beyond 36 months. The median overall survival from the time sunitinib was initiated was 26.7 months with a progression-free survival of 4.1 months (95% confidence interval = 1.4\u201311.0). Most patients who experienced a clinical benefit were carriers of SDHB mutations.<\/jats:p>\n <\/jats:sec>\n \n Conclusion:<\/jats:title>\n Sunitinib is associated with tumor size reduction, decreased [18F]FDG-PET\/CT uptake, disease stabilization, and hypertension improvement in some patients with progressive metastatic PHEO\/PGL. Prospective multi-institutional clinical trials are needed to determine the true benefits of sunitinib.<\/jats:p>\n <\/jats:sec>","DOI":"10.1210\/jc.2012-2356","type":"journal-article","created":{"date-parts":[[2012,9,11]],"date-time":"2012-09-11T02:09:22Z","timestamp":1347329362000},"page":"4040-4050","source":"Crossref","is-referenced-by-count":198,"title":["Treatment with Sunitinib for Patients with Progressive Metastatic Pheochromocytomas and Sympathetic Paragangliomas"],"prefix":"10.1210","volume":"97","author":[{"given":"Montserrat","family":"Ayala-Ramirez","sequence":"first","affiliation":[{"name":"Departments of Endocrine Neoplasia and Hormonal Disorders (M.A-R., M.A.H., M.E.C., S.G.W., C.J.), Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecile N.","family":"Chougnet","sequence":"additional","affiliation":[{"name":"Departments of Nuclear Medicine and Endocrine Tumors (C.N.C., S.L., C.C., D.D., E.B.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mouhammed Amir","family":"Habra","sequence":"additional","affiliation":[{"name":"Departments of Endocrine Neoplasia and Hormonal Disorders (M.A-R., M.A.H., M.E.C., S.G.W., C.J.), Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Lynn","family":"Palmer","sequence":"additional","affiliation":[{"name":"Biostatistics (J.L.P.), Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Leboulleux","sequence":"additional","affiliation":[{"name":"Departments of Nuclear Medicine and Endocrine Tumors (C.N.C., S.L., C.C., D.D., E.B.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria E.","family":"Cabanillas","sequence":"additional","affiliation":[{"name":"Departments of Endocrine Neoplasia and Hormonal Disorders (M.A-R., M.A.H., M.E.C., S.G.W., C.J.), Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Caramella","sequence":"additional","affiliation":[{"name":"Departments of Nuclear Medicine and Endocrine Tumors (C.N.C., S.L., C.C., D.D., E.B.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pete","family":"Anderson","sequence":"additional","affiliation":[{"name":"Pediatrics (P.A.), The University of Texas MD Anderson Cancer Center, Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abir","family":"Al Ghuzlan","sequence":"additional","affiliation":[{"name":"Pathology (A.A.G.), Institute Gustave-Roussy, F-94805 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven G.","family":"Waguespack","sequence":"additional","affiliation":[{"name":"Departments of Endocrine Neoplasia and Hormonal Disorders (M.A-R., M.A.H., M.E.C., S.G.W., C.J.), Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Desir\u00e9e","family":"Deandreis","sequence":"additional","affiliation":[{"name":"Departments of Nuclear Medicine and Endocrine Tumors (C.N.C., S.L., C.C., D.D., E.B.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Baudin","sequence":"additional","affiliation":[{"name":"Departments of Nuclear Medicine and Endocrine Tumors (C.N.C., S.L., C.C., D.D., E.B.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camilo","family":"Jimenez","sequence":"additional","affiliation":[{"name":"Departments of Endocrine Neoplasia and Hormonal Disorders (M.A-R., M.A.H., M.E.C., S.G.W., C.J.), Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","reference":[{"key":"2020071615531890700_B1","first-page":"802","article-title":"Occurence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979.","volume":"58","author":"Beard","year":"1983","journal-title":"Mayo Clin Proc"},{"key":"2020071615531890700_B2","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1210\/jc.2010-1946","article-title":"Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators.","volume":"96","author":"Ayala-Ramirez","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071615531890700_B3","article-title":"Pathology and genetics: tumours of endocrine organs (IARC WHO Classification of Tumours)","author":"DeLellis","year":"2004"},{"key":"2020071615531890700_B4","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1055\/s-0031-1299707","article-title":"Metastatic pheochromocytoma and paraganglioma: focus on therapeutics.","volume":"44","author":"Plouin","year":"2012","journal-title":"Horm Metab Res"},{"key":"2020071615531890700_B5","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1016\/0046-8177(90)90227-V","article-title":"Immunohistochemical expression of epidermal growth factor receptors in human adrenocortical carcinoma.","volume":"21","author":"Kamio","year":"1990","journal-title":"Hum Pathol"},{"key":"2020071615531890700_B6","doi-asserted-by":"crossref","first-page":"1235","DOI":"10.1016\/S0002-9440(10)64400-8","article-title":"Angiogenesis and vascular architecture in pheochromocytomas: distinctive traits in malignant tumors.","volume":"161","author":"Favier","year":"2002","journal-title":"Am J Pathol"},{"key":"2020071615531890700_B7","doi-asserted-by":"crossref","first-page":"1056","DOI":"10.1067\/msy.2002.128613","article-title":"VEGF-mediated angiogenesis of human pheochromocytomas is associated to malignancy and inhibited by anti-VEGF antibodies in experimental tumors.","volume":"132","author":"Zielke","year":"2002","journal-title":"Surgery"},{"key":"2020071615531890700_B8","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1034\/j.1600-0463.2003.1110402.x","article-title":"VEGF in 105 pheochromocytomas: enhanced expression correlates with malignant outcome.","volume":"111","author":"Salmenkivi","year":"2003","journal-title":"APMIS"},{"key":"2020071615531890700_B9","doi-asserted-by":"crossref","first-page":"4162","DOI":"10.1200\/JCO.2008.21.3496","article-title":"Phase II study of high-dose [131I]metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma.","volume":"27","author":"Gonias","year":"2009","journal-title":"J Clin Oncol"},{"key":"2020071615531890700_B10","doi-asserted-by":"crossref","first-page":"8812","DOI":"10.1200\/JCO.2005.03.1484","article-title":"Genetic testing in pheochromocytoma or functional paraganglioma.","volume":"23","author":"Amar","year":"2005","journal-title":"J Clin Oncol"},{"key":"2020071615531890700_B11","doi-asserted-by":"crossref","first-page":"680","DOI":"10.1055\/s-0029-1214381","article-title":"Malignant pheochromocytomas and paragangliomas: molecular signaling pathways and emerging therapies.","volume":"41","author":"Santarpia","year":"2009","journal-title":"Horm Metab Res"},{"key":"2020071615531890700_B12","doi-asserted-by":"crossref","first-page":"884","DOI":"10.1200\/JCO.2006.06.3602","article-title":"Sunitinib: from rational design to clinical efficacy.","volume":"25","author":"Chow","year":"2007","journal-title":"J Clin Oncol"},{"key":"2020071615531890700_B13","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1210\/jc.2008-1972","article-title":"Use of the tyrosine kinase inhibitor sunitinib in a patient with von Hippel-Lindau disease: targeting angiogenic factors in pheochromocytoma and other von Hippel-Lindau disease-related tumors.","volume":"94","author":"Jimenez","year":"2009","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071615531890700_B14","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1200\/JCO.2008.19.9380","article-title":"Patient with malignant paraganglioma responding to the multikinase inhibitor sunitinib malate.","volume":"27","author":"Hahn","year":"2009","journal-title":"J Clin Oncol"},{"key":"2020071615531890700_B15","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1210\/jc.2008-1836","article-title":"Rationale and evidence for sunitinib in the treatment of malignant paraganglioma\/pheochromocytoma.","volume":"94","author":"Joshua","year":"2009","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071615531890700_B16","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1016\/j.ejca.2008.10.026","article-title":"New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1).","volume":"45","author":"Eisenhauer","year":"2009","journal-title":"Eur J Cancer"},{"key":"2020071615531890700_B17","doi-asserted-by":"crossref","first-page":"122S","DOI":"10.2967\/jnumed.108.057307","article-title":"From RECIST to PERCIST: evolving considerations for PET response criteria in solid tumors.","volume":"50","author":"Wahl","year":"2009","journal-title":"J Nucl Med"},{"key":"2020071615531890700_B18","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1093\/jjco\/hyp005","article-title":"Sunitinib, a novel therapy for anthracycline- and cisplatin-refractory malignant pheochromocytoma.","volume":"39","author":"Park","year":"2009","journal-title":"Jpn J Clin Oncol"},{"key":"2020071615531890700_B19","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1371\/journal.pgen.0010008","article-title":"A HIF1\u03b1 regulatory loop links hypoxia and mitochondrial signals in pheochromocytomas.","volume":"1","author":"Dahia","year":"2005","journal-title":"PLoS Genet"},{"key":"2020071615531890700_B20","doi-asserted-by":"crossref","first-page":"4593","DOI":"10.1210\/jc.2006-0920","article-title":"Expression of HIF-1\u03b1, HIF-2\u03b1 (EPAS1), and their target genes in paraganglioma and pheochromocytoma with VHL and SDH mutations.","volume":"91","author":"Pollard","year":"2006","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071615531890700_B21","doi-asserted-by":"crossref","first-page":"2023","DOI":"10.1210\/jc.2009-2830","article-title":"A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma.","volume":"95","author":"Waguespack","year":"2010","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071615531890700_B22","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1111\/j.1365-2265.2007.03086.x","article-title":"Mutations associated with succinate dehydrogenase D-related malignant paragangliomas.","volume":"68","author":"Timmers","year":"2008","journal-title":"Clin Endocrinol (Oxf)"},{"key":"2020071615531890700_B23","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/j.ccr.2005.06.015","article-title":"Neuronal apoptosis linked to EglN3 prolyl hydroxylase and familial pheochromocytoma genes: developmental culling and cancer.","volume":"8","author":"Lee","year":"2005","journal-title":"Cancer Cell"},{"key":"2020071615531890700_B24","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1007\/s00345-010-0519-5","article-title":"Update on systemic therapies of metastatic renal cell 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