{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T11:00:02Z","timestamp":1789902002821,"version":"4.0.1"},"reference-count":0,"publisher":"Georg Thieme Verlag KG","issue":"02\/03","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Exp Clin Endocrinol Diabetes"],"published-print":{"date-parts":[[2019,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Phaeochromocytomas (PCC) and paragangliomas (PGL) are rare tumours arising from the chromaffin cells of the adrenal medulla (PCC) or the paraganglia located outside the adrenal gland (PGL). However, their incidence is likely to be underestimated; around 10% of all PCC\/PGL are metastatic, with higher metastatic potential of PGLs compared to PCCs. If benign, surgery is the treatment of choice, but if metastatic, therapy is challenging. Here we review the currently existing therapy options for metastatic PCCs\/PGLs including conventional chemotherapy (the original Averbuch scheme, but updated), radiopharmaceutical treatments (131I-MIBG, 90Y- and 177Lu-DOTATATE) and novel targeted therapies (anti-angiogenic tyrosine kinase inhibitors and mTORC1 inhibitors), emphasising future therapeutic approaches (HIF-2\u03b1 and PARP inhibitors, temozolomide alone, metronomic temozolomide, somatostatin analogues) based on the oncogenic signalling pathways related to three different clusters comprising more than 20 well-characterised PCC\/PGL susceptibility genes. We suggest that targeted combination therapies including repurposed agents may offer more effective future options worthy of exploration.<\/jats:p>","DOI":"10.1055\/a-0715-1888","type":"journal-article","created":{"date-parts":[[2018,9,20]],"date-time":"2018-09-20T22:55:32Z","timestamp":1537484132000},"page":"117-128","source":"Crossref","is-referenced-by-count":43,"title":["Metastatic Phaeochromocytoma: Spinning Towards More Promising Treatment Options"],"prefix":"10.1055","volume":"127","author":[{"given":"Svenja","family":"N\u00f6lting","sequence":"additional","affiliation":[{"name":"Medizinische Klinik und Poliklinik IV, Interdisciplinary Center of Neuroendocrine Tumours of the GastroEnteroPancreatic System (GEPNET-KUM), Klinikum der Universit\u00e4t M\u00fcnchen (KUM), Ludwig-Maximilians-University, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley","family":"Grossman","sequence":"additional","affiliation":[{"name":"Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, Royal Free Hospital ENETS Centre of Excellence, London, and Barts and the London Scool of Medicine, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karel","family":"Pacak","sequence":"additional","affiliation":[{"name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2018,9,20]]},"container-title":["Experimental and Clinical Endocrinology &amp; Diabetes"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-0715-1888.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,2,27]],"date-time":"2019-02-27T23:48:36Z","timestamp":1551311316000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-0715-1888"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9,20]]},"references-count":0,"journal-issue":{"issue":"02\/03","published-online":{"date-parts":[[2019,2,27]]},"published-print":{"date-parts":[[2019,2]]}},"URL":"https:\/\/doi.org\/10.1055\/a-0715-1888","relation":{},"ISSN":["0947-7349","1439-3646"],"issn-type":[{"value":"0947-7349","type":"print"},{"value":"1439-3646","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,9,20]]}}}