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The vast majority of lesions are sporadic in nature, approximately 85% of which are parathyroid adenomas, while hyperplasia and carcinoma account for 10\u201315% and fewer than 1%, of cases, respectively. Heritable forms of <jats:styled-content style=\"fixed-case\">PHPT<\/jats:styled-content> are much less common and present challenges both to clinicians and pathologists, particularly when they are the presenting feature of an endocrine syndrome. In such instances, pathologists play a key role in alerting physicians to the possibility of an underlying heritable endocrine syndrome and the potential for extra\u2010endocrine manifestations. Therefore, a working knowledge of these disorders is essential for providing guidance to treating physicians. The aim of this update is to review the clinicopathological features, genetic bases and current management for patients with <jats:styled-content style=\"fixed-case\">PHPT<\/jats:styled-content> associated with multiple endocrine neoplasia (<jats:styled-content style=\"fixed-case\">MEN<\/jats:styled-content>) types 1, 2A and 4 and hyperparathyroidism\u2010jaw tumour (<jats:styled-content style=\"fixed-case\">HPT<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">JT<\/jats:styled-content>) syndrome in the context of the 2017 World Health Organization (<jats:styled-content style=\"fixed-case\">WHO<\/jats:styled-content>) Classification of Tumours of the Endocrine Organs. Additionally, familial isolated hyperparathyroidism, familial hypocalciuric hypercalcaemia and neonatal severe hyperparathyroidism are discussed.<\/jats:p>","DOI":"10.1111\/his.13306","type":"journal-article","created":{"date-parts":[[2017,12,14]],"date-time":"2017-12-14T06:41:17Z","timestamp":1513233677000},"page":"117-132","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["Heritable forms of primary hyperparathyroidism: a current perspective"],"prefix":"10.1111","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7137-948X","authenticated-orcid":false,"given":"Ronald A","family":"DeLellis","sequence":"first","affiliation":[{"name":"Rhode Island Hospital and Alpert School of Medicine of Brown University Providence RI USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8999-9890","authenticated-orcid":false,"given":"Shamlal","family":"Mangray","sequence":"additional","affiliation":[{"name":"Rhode Island Hospital and Alpert School of Medicine of Brown University Providence RI USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,12,14]]},"reference":[{"issue":"2","key":"e_1_2_10_2_1","first-page":"N37","article-title":"Hyperparathyroidism in hereditary syndromes: special expressions and special managements","volume":"17","author":"Marx SJ","year":"2002","journal-title":"J. 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