{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T09:36:03Z","timestamp":1789983363324,"version":"4.0.1"},"reference-count":81,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,12,14]],"date-time":"2017-12-14T00:00:00Z","timestamp":1513209600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2018,1]]},"abstract":"<jats:p>Phaeochromocytoma and paraganglioma (<jats:styled-content style=\"fixed-case\">PHEO<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">PGL<\/jats:styled-content>) are rare tumours with an estimated annual incidence of 3 per million. Advances in molecular understanding have led to the recognition that at least 30\u201340% arise in the setting of hereditary disease. Germline mutations in the succinate dehydrogenase genes <jats:italic><jats:styled-content style=\"fixed-case\">SDHA<\/jats:styled-content><\/jats:italic>,<jats:italic> <jats:styled-content style=\"fixed-case\">SDHB<\/jats:styled-content><\/jats:italic>,<jats:italic> <jats:styled-content style=\"fixed-case\">SDHC<\/jats:styled-content><\/jats:italic>,<jats:italic> <jats:styled-content style=\"fixed-case\">SDHD<\/jats:styled-content><\/jats:italic> and <jats:italic><jats:styled-content style=\"fixed-case\">SDHAF<\/jats:styled-content>2<\/jats:italic> are the most prevalent of the more than 19 hereditary genetic abnormalities which have been reported. It is therefore recommended that, depending on local resources and availability, at least some degree of genetic testing should be offered to all <jats:styled-content style=\"fixed-case\">PHEO<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">PGL<\/jats:styled-content> patients, including those with clinically sporadic disease. It is now accepted that that all <jats:styled-content style=\"fixed-case\">PHEO<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">PGL<\/jats:styled-content> have some metastatic potential; therefore, concepts of benign and malignant <jats:styled-content style=\"fixed-case\">PHEO<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">PGL<\/jats:styled-content> have no meaning and have been replaced by a risk stratification approach. Although there is broad acceptance that certain features, including high proliferative activity, invasive growth, increased cellularity, large tumour nests and comedonecrosis, are associated with an increased risk of metastasis, it remains difficult to predict the clinical behaviour of individual tumours and no single risk stratification scheme is endorsed or in widespread use. In this review, we provide an update on advances in the pathology and genetics of <jats:styled-content style=\"fixed-case\">PHEO<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">PGL<\/jats:styled-content> with an emphasis on the changes introduced in the <jats:styled-content style=\"fixed-case\">WHO<\/jats:styled-content> 2017 classification of endocrine neoplasia relevant to practising surgical pathologists.<\/jats:p>","DOI":"10.1111\/his.13402","type":"journal-article","created":{"date-parts":[[2017,12,14]],"date-time":"2017-12-14T06:41:17Z","timestamp":1513233677000},"page":"97-105","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":137,"title":["Pathology and genetics of phaeochromocytoma and paraganglioma"],"prefix":"10.1111","volume":"72","author":[{"given":"John","family":"Turchini","sequence":"first","affiliation":[{"name":"Cancer Diagnosis and Pathology Group Kolling Institute of Medical Research Royal North Shore Hospital St Leonards NSW Australia"},{"name":"University of Sydney Sydney NSW Australia"},{"name":"Department of Anatomical Pathology NSW Health Pathology Royal North Shore Hospital St Leonards NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronica K Y","family":"Cheung","sequence":"additional","affiliation":[{"name":"Cancer Diagnosis and Pathology Group Kolling Institute of Medical Research Royal North Shore Hospital St Leonards NSW Australia"},{"name":"Department of Anatomical Pathology NSW Health Pathology Royal North Shore Hospital St Leonards NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthur S","family":"Tischler","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Tufts Medical Center Tufts University School of Medicine Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald R","family":"De Krijger","sequence":"additional","affiliation":[{"name":"Department of Pathology Erasmus MC Cancer Institute University Medical Center Rotterdam Rotterdam The Netherlands"},{"name":"Department of Pathology Reinier de Graaf Hospital Delft The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony J","family":"Gill","sequence":"additional","affiliation":[{"name":"Cancer Diagnosis and Pathology Group Kolling Institute of Medical Research Royal North Shore Hospital St Leonards NSW Australia"},{"name":"University of Sydney Sydney NSW Australia"},{"name":"Department of Anatomical Pathology NSW Health Pathology Royal North Shore Hospital St Leonards NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,12,14]]},"reference":[{"key":"e_1_2_10_2_1","first-page":"179","volume-title":"Pathology and genetics of endocrine organs","author":"Kloeppel G","year":"2017"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/hed.23436"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1210\/jcem.86.11.8034"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.23360"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000534"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2796.2009.02111.x"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAT.0b013e3283539932"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.13277"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2014-3399"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2015-103218"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddr324"},{"key":"e_1_2_10_13_1","first-page":"187","article-title":"The incidence rate of phaeochromocytoma and Conn's syndrome in Denmark, 1977\u20131981","volume":"2","author":"Andersen GS","year":"1988","journal-title":"J. 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