{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T08:08:15Z","timestamp":1789546095617,"version":"build-2803163510"},"reference-count":22,"publisher":"The Endocrine Society","issue":"10","license":[{"start":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T00:00:00Z","timestamp":1789516800000},"content-version":"vor","delay-in-days":13,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100020950","name":"National Science and Technology Council","doi-asserted-by":"publisher","award":["114-2314-B-002-064-MY3"],"award-info":[{"award-number":["114-2314-B-002-064-MY3"]}],"id":[{"id":"10.13039\/501100020950","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005762","name":"National Taiwan University Hospital","doi-asserted-by":"publisher","award":["115-N0044"],"award-info":[{"award-number":["115-N0044"]}],"id":[{"id":"10.13039\/501100005762","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,9,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare, preinvasive lung disorder characterized by multifocal neuroendocrine cell proliferation. Because it presents with chronic cough and wheezing, it is frequently misdiagnosed as asthma. Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) arising from DIPNECH is exceptionally rare. We present a rare case of a 62-year-old woman with a long-standing history of presumed asthma who developed ACTH-dependent Cushing syndrome. Bilateral inferior petrosal sinus sampling confirmed ectopic ACTH secretion. Subsequently, comprehensive thoracic imaging revealed a potential ectopic source in a dominant right middle lobe nodule, accompanied by subtle micronodules and mosaic attenuation, which are characteristic of DIPNECH. Targeted lobectomy achieved complete remission, yielding a histopathological diagnosis of a neuroendocrine tumor within background DIPNECH that demonstrated marked immunohistochemical ACTH heterogeneity among hyperplastic cells and tumorlets. Consistent with previously reported cases, our findings highlight that even subtle pulmonary nodules can secrete ACTH and must be evaluated in unexplained EAS. Furthermore, because these subcentimeter nodules possess secretory potential and morphological progression risks, lifelong multidisciplinary surveillance is imperative.<\/jats:p>","DOI":"10.1210\/jcemcr\/luag226","type":"journal-article","created":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T11:50:38Z","timestamp":1786017038000},"source":"Crossref","is-referenced-by-count":0,"title":["Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with ectopic adrenocorticotropic hormone syndrome in Asia"],"prefix":"10.1210","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-6219-2198","authenticated-orcid":false,"given":"Wei-Lin","family":"Huang","sequence":"first","affiliation":[{"name":"Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital , Taipei City 100225 ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min-Shu","family":"Hsieh","sequence":"additional","affiliation":[{"name":"Department of Pathology, National Taiwan University Hospital , Taipei City 100225 ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuan-Yu","family":"Tsai","sequence":"additional","affiliation":[{"name":"Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Hsin-Chu Branch , Hsin-Chu City 302058 ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1268-3729","authenticated-orcid":false,"given":"Mong-Wei","family":"Lin","sequence":"additional","affiliation":[{"name":"Division of Thoracic Surgery, Department of Surgery, National Taiwan University Hospital , Taipei City 100225 ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ching-Kai","family":"Lin","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, National Taiwan University Cancer Center , Taipei City 106037 ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8995-7420","authenticated-orcid":false,"given":"Chia-Hung","family":"Lin","sequence":"additional","affiliation":[{"name":"Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital , Taipei City 100225 ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2026,9,16]]},"reference":[{"key":"2026091603530963100_luag226-B1","first-page":"130","volume-title":"Thoracic Tumours","author":"Rossi","year":"2021","edition":"5th 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tumour","volume":"59","author":"Sun","year":"2022","journal-title":"Eur Respir J"},{"issue":"13","key":"2026091603530963100_luag226-B5","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1056\/NEJM199809243391304","article-title":"Cushing's syndrome caused by corticotropin secretion by pulmonary tumorlets","volume":"339","author":"Arioglu","year":"1998","journal-title":"N Engl J Med"},{"issue":"10","key":"2026091603530963100_luag226-B6","doi-asserted-by":"crossref","DOI":"10.1111\/jne.13184","article-title":"Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH): prevalence, clinicopathological characteristics and survival outcome in a cohort of 311 patients with well-differentiated lung neuroendocrine tumours","volume":"34","author":"Hayes","year":"2022","journal-title":"J Neuroendocrinol"},{"key":"2026091603530963100_luag226-B7","author":"Ferreira"},{"issue":"997","key":"2026091603530963100_luag226-B8","doi-asserted-by":"crossref","first-page":"e14","DOI":"10.1259\/bjr\/91375895","article-title":"Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an unusual cause of cyclical ectopic adrenocorticotrophic syndrome","volume":"84","author":"Cameron","year":"2011","journal-title":"British Journal of Radiology"},{"key":"2026091603530963100_luag226-B9","author":"Piazza"},{"issue":"8","key":"2026091603530963100_luag226-B10","article-title":"Ectopic Cushing syndrome secondary to diffuse idiopathic neuroendocrine cell hyperplasia-A report of 2 cases","volume":"2","author":"Lopez Fanas","year":"2024","journal-title":"JCEM Case Rep"},{"key":"2026091603530963100_luag226-B11","author":"Machenahalli"},{"issue":"8","key":"2026091603530963100_luag226-B12","article-title":"Ectopic Cushing's syndrome in a patient with DIPNECH and metastatic lung carcinoid","volume":"72","author":"Ribeiro","year":"2025","journal-title":"Endocrinol Diabetes Nutr (Engl Ed)"},{"key":"2026091603530963100_luag226-B13","author":"Ali"},{"issue":"1","key":"2026091603530963100_luag226-B14","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1007\/s12022-022-09708-2","article-title":"Overview of the 2022 WHO classification of neuroendocrine neoplasms","volume":"33","author":"Rindi","year":"2022","journal-title":"Endocr Pathol"},{"issue":"2","key":"2026091603530963100_luag226-B15","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1378\/chest.14-0711","article-title":"The clinical course of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia","volume":"147","author":"Carr","year":"2015","journal-title":"CHEST"},{"issue":"3","key":"2026091603530963100_luag226-B16","doi-asserted-by":"crossref","first-page":"900","DOI":"10.1007\/s00330-015-3882-1","article-title":"Comparison of diagnostic accuracy of (111)In-pentetreotide SPECT and 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