{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T21:48:13Z","timestamp":1769204893623,"version":"3.49.0"},"reference-count":17,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,3,29]],"date-time":"2021-03-29T00:00:00Z","timestamp":1616976000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2021,3,29]],"date-time":"2021-03-29T00:00:00Z","timestamp":1616976000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Med Case Reports"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Pheochromocytomas are a subset of paragangliomas, which are a rare group of neural crest cell-derived tumors. Malignant cases of both pheochromocytomas and paragangliomas are even rarer, and currently there is no standard of care. This case report details the use of off-label immunotherapy and its efficacy in the management of the aforementioned tumor.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>Herein is presented a case of a 60-year-old Caucasian female with a rare malignant pheochromocytoma. The tumor was determined to be unresectable because of involvement of surrounding organs. Radiation therapy was also not a viable option because of concerns over appreciable toxicity in relation to mass size. As there is no standard of care for malignant cases, the patient was started on chemotherapeutic agents but was soon shown to be intolerant to this treatment. As she was ineligible for several clinical trials, the patient was started on the off-label immunotherapeutic agents nivolumab and ipilimumab. Immunotherapy use resulted in decreased tumor size, improved quality of life, and reconsideration for radiation therapy.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>The use of immunotherapy in pheochromocytoma in this patient clearly demonstrated substantial benefit, as she was able to be reconsidered for radiation therapy. Not only has the patient been tolerant of this treatment, but she has exhibited progression-free survival of over 20\u00a0months. As there is no current standard treatment for malignant pheochromocytomas, the success of its use in this patient lends support to the ongoing clinical trials regarding the use of immunotherapy in rare tumors, including pheochromocytomas.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13256-021-02733-5","type":"journal-article","created":{"date-parts":[[2021,3,28]],"date-time":"2021-03-28T23:03:05Z","timestamp":1616972585000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["The use of immunotherapy treatment in malignant pheochromocytomas\/paragangliomas: a case report"],"prefix":"10.1186","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9926-6690","authenticated-orcid":false,"given":"Robin Raquel","family":"Rodriguez","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saleha","family":"Rizwan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Khaled","family":"Alhamad","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gene Grant","family":"Finley","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,3,29]]},"reference":[{"key":"2733_CR1","unstructured":"Pheochromocytoma and paraganglioma: types of treatment [Internet]. Cancer.Net. 2020. https:\/\/www.cancer.net\/cancer-types\/pheochromocytoma-and-paraganglioma\/types-treatment. Accessed Sep 2020."},{"issue":"9","key":"2733_CR2","doi-asserted-by":"publisher","first-page":"687","DOI":"10.1055\/s-0029-1231025","volume":"41","author":"R Adjalle","year":"2009","unstructured":"Adjalle R, Plouin PF, Pacak K, et\u00a0al. Treatment of malignant pheochromocytoma. Horm Metab Res. 2009;41(9):687\u201396.","journal-title":"Horm Metab Res"},{"key":"2733_CR3","unstructured":"Treatment of pheochromocytoma in adults [Internet]. UpToDate. 2019. https:\/\/www.uptodate.com\/contents\/treatment-of-pheochromocytoma-in-adults. Accessed Aug 2020."},{"key":"2733_CR4","unstructured":"Grossman A. Pheochromocytoma [Internet]. Merck Manual. 2019. https:\/\/www.merckmanuals.com\/professional\/endocrine-and-metabolic-disorders\/adrenal-disorders\/pheochromocytoma. Accessed Aug 2020."},{"issue":"277","key":"2733_CR5","first-page":"1","volume":"9","author":"C Jimenez","year":"2018","unstructured":"Jimenez C. Treatment for patients with malignant pheochromocytomas and paragangliomas: a perspective from the hallmarks of cancer. Front Endocrinol. 2018;9(277):1\u201312.","journal-title":"Front Endocrinol."},{"issue":"10","key":"2733_CR6","doi-asserted-by":"publisher","first-page":"2290","DOI":"10.1158\/1078-0432.CCR-19-3356","volume":"26","author":"SP Patel","year":"2020","unstructured":"Patel SP, Othus M, Chae YK, et\u00a0al. A phase II basket trial of dual anti-CTLA-4 and anti-PD-1 blockade in rare tumors (DART SWOG 1609) in patients with nonpancreatic neuroendocrine tumors. Clin Cancer Res. 2020;26(10):2290\u20136.","journal-title":"Clin Cancer Res."},{"key":"2733_CR7","unstructured":"Nivolumab and ipilimumab in treating patients with rare tumors [Internet]. Cancer.gov. https:\/\/www.cancer.gov\/about-cancer\/treatment\/clinical-trials\/search\/v?id=NCI-2016-01041&r=1. Accessed Sep 2020."},{"key":"2733_CR8","doi-asserted-by":"publisher","DOI":"10.1136\/jitc-2019-000347corr1","author":"A Naing","year":"2020","unstructured":"Naing A, Meric-Bernstam F, Stephen B, et\u00a0al. Phase 2 study of pembrolizumab in patients with advanced rare cancers. J ImmunoTher Cancer. 2020. https:\/\/doi.org\/10.1136\/jitc-2019-000347corr1.","journal-title":"J ImmunoTher Cancer."},{"key":"2733_CR9","doi-asserted-by":"publisher","DOI":"10.1016\/j.nucmedbio.2008.04.007","author":"I Ilias","year":"2008","unstructured":"Ilias I, Pacek K. A clinical overview of pheochromocytomas\/paragangliomas and carcinoid tumors. Nucl Med Biol. 2008. https:\/\/doi.org\/10.1016\/j.nucmedbio.2008.04.007.","journal-title":"Nucl Med Biol."},{"issue":"4","key":"2733_CR10","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1016\/j.eucr.2014.05.001","volume":"2","author":"MH Johnson","year":"2014","unstructured":"Johnson MH, Cavallo JA, Figenshau RS. Malignant and metastatic pheochromocytoma: case report and review of the literature. Urol Case Rep. 2014;2(4):139\u201341.","journal-title":"Urol Case Rep."},{"key":"2733_CR11","unstructured":"Young W, Elfiky A. Paraganglioma and pheochromocytoma: management of malignant disease. [Internet] UpToDate. 2019. https:\/\/www.uptodate.com\/contents\/paraganglioma-and-pheochromocytoma-management-of-malignant-disease?search=pheochromocytoma%20treatment&topicRef=142&source=see_link#H28826173. Accessed Aug 2020."},{"key":"2733_CR12","doi-asserted-by":"publisher","DOI":"10.1530\/endoabs.32.P216","author":"A Gruszka","year":"2013","unstructured":"Gruszka A, Zieleniewski W, Kotecka-Blicharz A, et\u00a0al. Long-term treatment with octreotide in a patient with malignant pheochromocytoma: impact on survival and time to tumor progression. Endocrine Abstracts. 2013. https:\/\/doi.org\/10.1530\/endoabs.32.P216.","journal-title":"Endocrine Abstracts."},{"key":"2733_CR13","unstructured":"Lu-177-DOTATATE (Lutathera) in therapy of inoperable pheochromocytoma\/ paraganglioma. [Internet] NIH. 2017. https:\/\/clinicaltrials.gov\/ct2\/show\/NCT03206060. Accessed 2 Sep 2020."},{"key":"2733_CR14","doi-asserted-by":"publisher","DOI":"10.12688\/f1000research.13995.1","author":"R Toledo","year":"2018","unstructured":"Toledo R, Jimenez C. Recent advances in the management of malignant pheochromocytoma and paraganglioma: focus on tyrosine kinase and hypoxia-inducible factor inhibitors. F1000 Res. 2018. https:\/\/doi.org\/10.12688\/f1000research.13995.1.","journal-title":"F1000 Res."},{"key":"2733_CR15","doi-asserted-by":"publisher","first-page":"1050","DOI":"10.1038\/modpathol.2013.233","volume":"27","author":"C Cassol","year":"2014","unstructured":"Cassol C, Winer D, Liu W, et\u00a0al. Tyrosine kinase receptors as molecular targets in pheochromocytomas and paragangliomas. Mod Pathol. 2014;27:1050\u201362.","journal-title":"Mod Pathol"},{"key":"2733_CR16","unstructured":"Lenvatinib in treating patients with metastatic or advanced pheochromocytoma or paraganglioma that cannot be removed by surgery [Internet]. https:\/\/clinicaltrials.gov\/ct2\/show\/study\/NCT0300836. Accessed Sep 2020."},{"key":"2733_CR17","unstructured":"Schilsky R. Tumor-agnostic treatment for cancer: an expert perspective. [Internet] Cancer.net. 2018. https:\/\/www.cancer.net\/blog\/2018-12\/tumor-agnostic-treatment-cancer-expert-perspective. Accessed Aug 2020."}],"container-title":["Journal of Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-021-02733-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s13256-021-02733-5\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-021-02733-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,3,28]],"date-time":"2021-03-28T23:06:00Z","timestamp":1616972760000},"score":1,"resource":{"primary":{"URL":"https:\/\/jmedicalcasereports.biomedcentral.com\/articles\/10.1186\/s13256-021-02733-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,29]]},"references-count":17,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["2733"],"URL":"https:\/\/doi.org\/10.1186\/s13256-021-02733-5","relation":{},"ISSN":["1752-1947"],"issn-type":[{"value":"1752-1947","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,3,29]]},"assertion":[{"value":"24 November 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 February 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 March 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Verbal and written consent were obtained from the patient.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors have no conflicts of interest or competing interests to report","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"172"}}