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Approximately 20% of hereditary\n                    <jats:styled-content style=\"fixed-case\">PPGL<\/jats:styled-content>\n                    are caused by pathogenic germ line variants in genes of the succinate dehydrogenase complex (\n                    <jats:styled-content style=\"fixed-case\">SDH<\/jats:styled-content>\n                    x),\n                    <jats:italic>TMEM127<\/jats:italic>\n                    or\n                    <jats:italic>MAX<\/jats:italic>\n                    . Herein we present guidelines regarding genetic testing of family members and their surveillance based on a thorough literature review. All cases of\n                    <jats:styled-content style=\"fixed-case\">PPGL<\/jats:styled-content>\n                    are recommended genetic testing for germ line variants regardless of patient and family characteristics. At minimum,\n                    <jats:italic>FH, NF1, RET, SDHB, SDHD<\/jats:italic>\n                    and\n                    <jats:italic>VHL<\/jats:italic>\n                    should be tested. In addition, testing of\n                    <jats:italic>\n                      MEN1,\n                      <jats:styled-content style=\"fixed-case\">SDHA<\/jats:styled-content>\n                      ,\n                      <jats:styled-content style=\"fixed-case\">SDHAF<\/jats:styled-content>\n                      2,\n                      <jats:styled-content style=\"fixed-case\">SDHC<\/jats:styled-content>\n                      ,\n                      <jats:styled-content style=\"fixed-case\">TMEM<\/jats:styled-content>\n                      127\n                    <\/jats:italic>\n                    and\n                    <jats:italic>MAX<\/jats:italic>\n                    is recommended. Healthy first\u2010degree relatives (and second\u2010degree relatives in the case of\n                    <jats:italic>SDHD<\/jats:italic>\n                    and\n                    <jats:italic>SDHAF2<\/jats:italic>\n                    which are maternally imprinted) should be offered carrier testing. Carriers of pathogenic variants should be offered surveillance with annual biochemical measurements of methoxy\u2010catecholamines and bi\u2010annual rapid whole\u2010body magnetic resonance imaging and clinical examination. Surveillance should start 5\u00a0years before the earliest age of onset in the family and thus only children eligible for surveillance should be offered pre\u2010symptomatic genetic testing. The surveillance of children younger than 15\u00a0years needs to be individually designed. Our guidelines will provide a framework for patient management with the possibility to follow outcome via national registries and\/or follow\u2010up studies. Together with improved insights into the disease, this may enable optimisation of the surveillance scheme in order to minimise both anxiety and medical complications while ensuring early disease detection.\n                  <\/jats:p>","DOI":"10.1111\/joim.12869","type":"journal-article","created":{"date-parts":[[2018,12,8]],"date-time":"2018-12-08T08:29:02Z","timestamp":1544257742000},"page":"187-204","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":118,"title":["Genetic testing and surveillance guidelines in hereditary pheochromocytoma and paraganglioma"],"prefix":"10.1111","volume":"285","author":[{"given":"A.","family":"Muth","sequence":"first","affiliation":[{"name":"Department of Surgery Institute of Clinical Sciences Sahlgrenska Academy at the University of Gothenburg Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Crona","sequence":"additional","affiliation":[{"name":"Department of Medical Sciences Uppsala University Uppsala Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"O.","family":"Gimm","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine Link\u00f6ping University Link\u00f6ping Sweden"},{"name":"Department of Surgery Link\u00f6ping University Link\u00f6ping Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Elmgren","sequence":"additional","affiliation":[{"name":"Clinical Chemistry Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Filipsson","sequence":"additional","affiliation":[{"name":"Endocrinology Sk\u00e5ne University Hospital Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Stenmark Askmalm","sequence":"additional","affiliation":[{"name":"Department of Clinical Genetics Division of Laboratory Medicine Office for Medical Services Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Sandstedt","sequence":"additional","affiliation":[{"name":"Clinical Chemistry Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Tengvar","sequence":"additional","affiliation":[{"name":"Department of Radiology Karolinska University Hospital Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6079-164X","authenticated-orcid":false,"given":"E.","family":"Tham","sequence":"additional","affiliation":[{"name":"Clinical Genetics Karolinska University Hospital Stockholm Sweden"},{"name":"Department of Molecular Medicine and Surgery Karolinska Institutet Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,1,15]]},"reference":[{"key":"e_1_2_15_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12022-017-9484-5"},{"key":"e_1_2_15_3_1","doi-asserted-by":"publisher","DOI":"10.1186\/1897-4287-4-4-169"},{"key":"e_1_2_15_4_1","doi-asserted-by":"publisher","DOI":"10.1530\/ERC-11-0170"},{"key":"e_1_2_15_5_1","doi-asserted-by":"publisher","DOI":"10.1530\/ERC-15-0268"},{"key":"e_1_2_15_6_1","doi-asserted-by":"publisher","DOI":"10.1210\/er.2017-00062"},{"key":"e_1_2_15_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.0954-6820.1986.tb02755.x"},{"key":"e_1_2_15_8_1","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1016\/S0025-6196(26)01464-3","article-title":"Prevalence of clinically unsuspected pheochromocytoma. 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