{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,7]],"date-time":"2026-03-07T20:11:20Z","timestamp":1772914280174,"version":"3.50.1"},"reference-count":38,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,1,22]],"date-time":"2024-01-22T00:00:00Z","timestamp":1705881600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Endocrinol."],"abstract":"<jats:p>Paediatric Cushing\u2019s disease (CD) is characterized by excess ACTH secretion from a pituitary adenoma, leading to hypercortisolism. It has approximately 5% of the incidence of adult CD and is a rare disorder in the paediatric age range. The four most specific presenting features of hypercortisolism are: change in facial appearance, weight gain, decreased linear growth and virilisation shown by advanced pubic hair for the stage of breast development or testicular volume. The main diagnostic priority is the demonstration of hypercortisolism followed by distinction between its ACTH-dependent and ACTH-independent origin, thus leading to identification of aetiology. All treatment options aim to resolve or control hypercortisolism. Consensus favours transsphenoidal (TSS) pituitary surgery with selective removal of the corticotroph adenoma. TSS in children with CD is now well established and induces remission in 70-100% of cases. External pituitary radiotherapy and bilateral adrenalectomy are second-line therapeutic approaches in subjects not responding to TSS. Long-term medical treatment is less frequently adopted. Recurrence in paediatric CD cases is low with factors predicting relapse being higher post-TSS cortisol and ACTH levels and rapid recovery of the hypothalamic-pituitary-adrenal axis after TSS. In summary, complete excision of the microadenoma with histological and biochemical evidence for this, predicts a low rate of recurrence of CD. Due to the need for rapid diagnosis and management to avoid the burden of prolonged exposure to hypercortisolism, tertiary university centres comprising both paediatric and adult endocrinology specialists together with experienced pituitary surgery and, eventually, radiotherapy units are recommended for referral of these patients.<\/jats:p>","DOI":"10.3389\/fendo.2024.1345174","type":"journal-article","created":{"date-parts":[[2024,1,22]],"date-time":"2024-01-22T04:30:26Z","timestamp":1705897826000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":7,"title":["Paediatric Cushing\u2019s disease: long-term outcome and predictors of recurrence"],"prefix":"10.3389","volume":"15","author":[{"given":"Martin O.","family":"Savage","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosario","family":"Ferrigno","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,1,22]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/s0140-6736(06)68699-6","article-title":"Cushing\u2019s syndrome","volume":"367","author":"Newell-Price","year":"2006","journal-title":"Lancet"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(14)61375-1","article-title":"Cushing\u2019s syndrome","volume":"386","author":"Lacroix","year":"2015","journal-title":"Lancet"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1007\/s11102-019-00951-1","article-title":"The incidence of Cushing\u2019s disease: a nationwide Swedish study","volume":"22","author":"Ragnarsson","year":"2019","journal-title":"Pituitary"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1007\/s11154-021-09626-4","article-title":"Paediatric Cushing\u2019s disease: epidemiology, pathogenesis, clinical management and outcome","volume":"22","author":"Ferrigno","year":"2021","journal-title":"Rev Endocr Metab Disord"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1210\/jcem.78.1.7507118","article-title":"Suppressed spontaneous and stimulated growth hormone secretion in patients with Cushing\u2019s disease before and after surgical cure","volume":"78","author":"Magiakou","year":"1994","journal-title":"J Clin Endocrinol Metab"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.4158\/EP10143.OR","article-title":"Cushing disease in children and adolescents: twenty years\u2019 experience in a tertiary care center in India","volume":"17","author":"Shah","year":"2011","journal-title":"Endocr Pract"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-10-1120","article-title":"Comparisons in the epidemiology, diagnostic features and cure rate by transsphenoidal surgery between paediatric and adult-onset Cushing\u2019s disease","volume":"164","author":"Storr","year":"2011","journal-title":"Eur J Endocrinol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"892","DOI":"10.1210\/jc.2012-3604","article-title":"Outcome of surgical treatment of 200 children with Cushing\u2019s disease","volume":"98","author":"Lonser","year":"2013","journal-title":"J Clin Endocrinol Metab"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-06-0588","article-title":"Long-term anterior pituitary function in patients with paediatric Cushing\u2019s disease treated with pituitary radiotherapy","volume":"156","author":"Chan","year":"2007","journal-title":"Eur J Endocrinol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2009-0302","article-title":"Postoperative testing to predict recurrent Cushing disease in children","volume":"94","author":"Batista","year":"2009","journal-title":"J Clin Endocrinol Metab"},{"key":"B11","doi-asserted-by":"publisher","first-page":"98","DOI":"10.1111\/cen.14131","article-title":"Abnormal linear growth in paediatric adrenal diseases: Pathogenesis, prevalence and management","volume":"92","author":"Minnetti","year":"2020","journal-title":"Clin Endocrinol (Oxf)"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2265.2007.02822.x","article-title":"Abnormal puberty in paediatric Cushing\u2019s disease; 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