{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T05:47:50Z","timestamp":1791870470491,"version":"4.3.4"},"reference-count":9,"publisher":"The Endocrine Society","issue":"11","license":[{"start":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T00:00:00Z","timestamp":1791849600000},"content-version":"vor","delay-in-days":18,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,9,25]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Primary adrenal neuroendocrine carcinoma (NEC), particularly the small cell subtype, is exceptionally rare. In patients with sarcoidosis, inflammatory lymphadenopathy may mimic a metastatic disease, making disease detection difficult. A 76-year-old man with sarcoidosis was referred for an enlarging left adrenal incidentaloma that had increased in size from 7\u2005mm to 19 \u00d7 14\u2005mm over 3 years. Hormonal evaluation revealed unremarkable findings; laparoscopic adrenalectomy was performed because malignancy could not be excluded. Histopathological examination revealed small cell NEC with positive synaptophysin and chromogranin A staining, and a Ki-67 labeling index of 80%. One month after surgery, para-aortic lymphadenopathy was detected. Fluorodeoxyglucose positron emission tomography\/computed tomography (CT) showed uptake in the para-aortic lymph nodes and ascending colon, where colonoscopy identified synchronous adenocarcinoma. The cause of the lymphadenopathy was uncertain because of coexisting sarcoidosis and colon cancer. Cisplatin plus irinotecan induced a partial response; however, disease progression was observed. CT-guided lymph node biopsy confirmed metastatic NEC. Second-line amrubicin therapy reduced tumor size; comprehensive genomic profiling revealed a tumor mutation-burden-high phenotype. This case highlights diagnostic challenges in the evaluation of lymphadenopathy in patients with sarcoidosis and synchronous malignancy, emphasizing the importance of pathological confirmation for guiding appropriate management.<\/jats:p>","DOI":"10.1210\/jcemcr\/luag313","type":"journal-article","created":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T11:32:21Z","timestamp":1791286341000},"source":"Crossref","is-referenced-by-count":0,"title":["Primary adrenal small cell neuroendocrine carcinoma in a patient with sarcoidosis and synchronous colon adenocarcinoma"],"prefix":"10.1210","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-1568-5668","authenticated-orcid":false,"given":"Asami","family":"Sano","sequence":"first","affiliation":[{"name":"Department of Diabetes, Endocrinology and Metabolism, Nagano Municipal Hospital , Nagano 381-0006 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tetsu","family":"Takeichi","sequence":"additional","affiliation":[{"name":"Department of Diabetes, Endocrinology and Metabolism, Nagano Municipal Hospital , Nagano 381-0006 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akio","family":"Miyamaoto","sequence":"additional","affiliation":[{"name":"Department of Diabetes, Endocrinology and Metabolism, Nagano Municipal Hospital , Nagano 381-0006 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yutaka","family":"Nishii","sequence":"additional","affiliation":[{"name":"Department of Diabetes, Endocrinology and Metabolism, Nagano Municipal Hospital , Nagano 381-0006 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teppei","family":"Yamamoto","sequence":"additional","affiliation":[{"name":"Department of Urology, Nagano Municipal Hospital , Nagano 381-0006 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukiko","family":"Kusama","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology, Nagano Municipal Hospital , Nagano 381-0006 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2026,10,13]]},"reference":[{"issue":"4","key":"2026101301314139900_luag313-B1","first-page":"196","article-title":"Small cell neuroendocrine carcinoma of the adrenal gland: a case report and review of the literature","volume":"2","author":"Tazi","year":"2011","journal-title":"World J Oncol"},{"key":"2026101301314139900_luag313-B2","author":"National Comprehensive Cancer Network"},{"issue":"1","key":"2026101301314139900_luag313-B3","doi-asserted-by":"crossref","first-page":"G1","DOI":"10.1093\/ejendo\/lvad066","article-title":"European Society of Endocrinology clinical practice guidelines on the management of adrenal incidentalomas, in collaboration with the European network for the study of adrenal tumors","volume":"189","author":"Fassnacht","year":"2023","journal-title":"Eur J Endocrinol"},{"issue":"6","key":"2026101301314139900_luag313-B4","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1056\/NEJMcp065470","article-title":"Clinical practice. The incidentally discovered adrenal mass","volume":"356","author":"Young","year":"2007","journal-title":"N Engl J Med"},{"issue":"2","key":"2026101301314139900_luag313-B5","first-page":"143","article-title":"18F-FDG PET in sarcoidosis: an observational study in 12 patients treated with infliximab","volume":"25","author":"Keijsers","year":"2008","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis"},{"issue":"10","key":"2026101301314139900_luag313-B6","first-page":"1571","article-title":"Comparative evaluation of FDG PET and gallium scintigraphy in patients with sarcoidosis","volume":"47","author":"Nishiyama","year":"2006","journal-title":"J Nucl Med"},{"issue":"6","key":"2026101301314139900_luag313-B7","doi-asserted-by":"crossref","first-page":"1949","DOI":"10.1378\/chest.07-1178","article-title":"Results of 188 whole-body fluorodeoxyglucose positron emission tomography scans in 137 patients with sarcoidosis","volume":"132","author":"Teirstein","year":"2007","journal-title":"Chest"},{"issue":"10","key":"2026101301314139900_luag313-B8","doi-asserted-by":"crossref","first-page":"1353","DOI":"10.1016\/S1470-2045(20)30445-9","article-title":"Association of tumour mutational burden with outcomes in patients with advanced solid tumors treated with pembrolizumab: prospective biomarker analysis of the multicohort, open-label, phase 2 KEYNOTE-158 study","volume":"21","author":"Marabelle","year":"2020","journal-title":"Lancet Oncol"},{"issue":"8","key":"2026101301314139900_luag313-B9","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0325727","article-title":"Evaluation of therapeutic agent selection based on comprehensive genomic profiling in gastroenteropancreatic neuroendocrine neoplasms","volume":"20","author":"Miyazawa","year":"2025","journal-title":"PLoS One"}],"container-title":["JCEM Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/4\/11\/luag313\/71891636\/luag313.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/4\/11\/luag313\/71891636\/luag313.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T05:31:50Z","timestamp":1791869510000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jcemcr\/article\/doi\/10.1210\/jcemcr\/luag313\/8911672"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,25]]},"references-count":9,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2026,9,25]]}},"URL":"https:\/\/doi.org\/10.1210\/jcemcr\/luag313","relation":{},"ISSN":["2755-1520"],"issn-type":[{"value":"2755-1520","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,11]]},"published":{"date-parts":[[2026,9,25]]},"article-number":"luag313"}}