{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T02:23:56Z","timestamp":1790821436965,"version":"4.1.0"},"reference-count":54,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2020,12,29]],"date-time":"2020-12-29T00:00:00Z","timestamp":1609200000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005642","name":"Universit\u00e0 degli Studi di Sassari","doi-asserted-by":"publisher","award":["SICT Project"],"award-info":[{"award-number":["SICT Project"]}],"id":[{"id":"10.13039\/501100005642","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Italian Ministry of Education, University and Research (MIUR)","award":["PRIN 2017Z3N3YC"],"award-info":[{"award-number":["PRIN 2017Z3N3YC"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Pheochromocytoma and paraganglioma are neuroendocrine neoplasms, originating in the adrenal medulla and in parasympathetic and sympathetic autonomic nervous system ganglia, respectively. They usually present as localized tumours curable with surgery. However, these tumours may exhibit heterogeneous clinical course, ranging from no\/minimal progression to aggressive (progressive\/metastatic) behavior. For this setting of patients, current therapies are unsatisfactory. Immune checkpoint inhibitors have shown outstanding results for several types of solid cancers. We therefore aimed to summarize and discuss available data on efficacy and safety of current FDA-approved immune checkpoint inhibitors in patients with pheochromocytoma and paraganglioma. After an extensive search, we found 15 useful data sources (four full-published articles, four supplements of scientific journals, seven ongoing registered clinical trials). The data we detected, even with the limit of the small number of patients treated, make a great expectation on the therapeutic use of immune checkpoint inhibitors. Besides, the newly detected predictors of response will (hopefully) be of great helps in selecting the subset of patients that might benefit the most from this class of drugs. Finally, new trials are in the starting blocks, and they are expected to shed in the next future new light on a therapy, which is considered a milestone in oncology.<\/jats:p>","DOI":"10.3390\/jcm10010088","type":"journal-article","created":{"date-parts":[[2020,12,29]],"date-time":"2020-12-29T10:04:46Z","timestamp":1609236286000},"page":"88","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Emerging Therapies in Pheochromocytoma and Paraganglioma: Immune Checkpoint Inhibitors in the Starting Blocks"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8367-5649","authenticated-orcid":false,"given":"Giuseppe","family":"Fanciulli","sequence":"first","affiliation":[{"name":"NET Unit, Department of Medical, Surgical and Experimental Sciences, University of Sassari\u2014Endocrine Unit, AOU Sassari, 07100 Sassari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3454-7330","authenticated-orcid":false,"given":"Sergio","family":"Di Molfetta","sequence":"additional","affiliation":[{"name":"Department of Emergency and Organ Transplantation, Section of Internal Medicine, Endocrinology, Andrology and Metabolic Diseases, University of Bari Aldo Moro, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0140-7297","authenticated-orcid":false,"given":"Andrea","family":"Dotto","sequence":"additional","affiliation":[{"name":"Endocrinology Unit, IRCCS Ospedale Policlinico San Martino, 16132 Genova, Italy"},{"name":"Department of Internal Medicine, University of Genova, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2394-996X","authenticated-orcid":false,"given":"Tullio","family":"Florio","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University of Genova, 16132 Genova, Italy"},{"name":"IRCCS Ospedale Policlinico San Martino, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiziana","family":"Feola","sequence":"additional","affiliation":[{"name":"Department of Clinical and Molecular Medicine, Sapienza University of Rome, 00161 Rome, Italy"},{"name":"Neuroendocrinology, Neuromed Institute, IRCCS, 86077 Pozzilli (IS), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manila","family":"Rubino","sequence":"additional","affiliation":[{"name":"Division of Gastrointestinal Medical Oncology and Neuroendocrine Tumors, European Institute of Oncology, IEO, IRCCS, 20141 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"de Cicco","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Endocrinology Unit, University Federico II, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annamaria","family":"Colao","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Endocrinology Unit, University Federico II, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9324-3946","authenticated-orcid":false,"given":"Antongiulio","family":"Faggiano","sequence":"additional","affiliation":[{"name":"Department of Clinical and Molecular Medicine, Sapienza University of Rome, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"NIKE Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,12,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1007\/s11912-013-0320-x","article-title":"Current and Future Treatments for Malignant Pheochromocytoma and Sympathetic Paraganglioma","volume":"15","author":"Jimenez","year":"2013","journal-title":"Curr. 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