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This class of neoplasms includes benign tumours and highly aggressive carcinomas. Adnexal tumours often present as solitary sporadic lesions, but can herald the presence of an inherited tumour syndrome such as Muir\u2013Torre syndrome, Cowden syndrome or CYLD cutaneous syndrome. In contrast to squamous cell carcinoma and basal cell carcinoma, molecular changes in adnexal neoplasia have been poorly characterised and there are few published reviews on the current state of knowledge.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>We reviewed findings in peer\u2010reviewed literature on molecular investigations of cutaneous adnexal tumours published to June 2021.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Recent discoveries have revealed diverse oncogenic drivers and tumour suppressor alterations in this class of tumours, implicating pathways including Ras\/MAPK, PI3K, YAP\/TAZ, beta\u2010catenin and nuclear factor kappa B (NF\u2010\u03baB). These observations have identified novel markers, such as NUT for poroma and porocarcinoma and PLAG1 for mixed tumours. Here, we provide a comprehensive overview and update of the molecular findings associated with adnexal tumours of the skin.<\/jats:p><\/jats:sec>","DOI":"10.1111\/his.14441","type":"journal-article","created":{"date-parts":[[2021,7,2]],"date-time":"2021-07-02T06:37:01Z","timestamp":1625207821000},"page":"166-183","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Molecular pathology of skin adnexal tumours"],"prefix":"10.1111","volume":"80","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2005-9988","authenticated-orcid":false,"given":"Jaclyn M","family":"Plotzke","sequence":"first","affiliation":[{"name":"Department of Pathology University of Michigan Ann Arbor MI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David J","family":"Adams","sequence":"additional","affiliation":[{"name":"Wellcome Sanger Institute Hinxton Cambridge UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0802-2883","authenticated-orcid":false,"given":"Paul W","family":"Harms","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Michigan Ann Arbor MI USA"},{"name":"Department of Dermatology University of Michigan Ann Arbor MI USA"},{"name":"Rogel Cancer Center University of Michigan Ann Arbor MI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,12,27]]},"reference":[{"key":"e_1_2_9_2_1","volume-title":"Cutaneous adnexal tumors","author":"Kazakov D","year":"2012"},{"key":"e_1_2_9_3_1","volume-title":"WHO classification of skin tumors","author":"Elder DE","year":"2018"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.path.2021.03.004"},{"key":"e_1_2_9_5_1","volume-title":"Dermatology","author":"Bolognia JL","year":"2018"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.path.2021.03.005"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10897-012-9552-4"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2005.00519.x"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-018-04008-y"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pathol.2016.05.004"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-18-1688"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.4759"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/mgg3.781"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.dad.0000190773.79463.75"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/DAD.0000000000001358"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/7747"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/cup.13113"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.0303-6987.2005.00267.x"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2010.99"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2020.05.121"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0560.2011.01836.x"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22061"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0230003"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13000-020-0925-y"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2014.11.002"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2007.02.015"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1523-1747.2003.12514.x"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-04-0307"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.0022-202X.2004.22321.x"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1038\/76006"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature01811"},{"issue":"2238","key":"e_1_2_9_32_1","first-page":"e6","article-title":"Malta (MYH9 associated elastin aggregation) syndrome: germline variants in MYH9 cause rare sweat duct proliferations and irregular elastin aggregations","volume":"139","author":"Fewings E","year":"2019","journal-title":"J. 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