{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T19:57:17Z","timestamp":1772308637452,"version":"3.50.1"},"reference-count":14,"publisher":"Walter de Gruyter GmbH","funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82200744"],"award-info":[{"award-number":["82200744"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"name":"the Health Science and Technology Development Special Foundation of Nanjing","award":["YKK22159"],"award-info":[{"award-number":["YKK22159"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec id=\"j_jpem-2025-0557_abs_001\">\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Ectopic ACTH syndrome (EAS) is a rare cause of Cushing\u2019s syndrome, especially in children, and has been only exceptionally linked to sacrococcygeal teratomas (SCTs). We report a pediatric case of SCT-associated EAS and review the literature to highlight diagnostic challenges and management strategies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"j_jpem-2025-0557_abs_002\">\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>A 13-year-old girl presented with hypertension, hypokalemia, hyperglycemia, and rapid weight gain. Biochemical testing confirmed ACTH-dependent hypercortisolism, while pituitary MRI was negative. Pelvic CT revealed a 5.1\u202fcm presacral mass. PET\/CT based on ^18F-FDG showed only mild glucose uptake (SUVmax 3.26) in the mass, whereas ^68Ga-DOTA-NOC PET\/CT demonstrated intense somatostatin receptor (SSTR) expression (SUVmax 55.86). Laparoscopic resection identified a mature teratoma containing a well-differentiated neuroendocrine tumor (NET G1) positive for ACTH and SSTR2. Postoperatively, cortisol\/ACTH levels normalized, and metabolic abnormalities resolved with 9-month follow-up showing no recurrence.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"j_jpem-2025-0557_abs_003\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This report describes the first adolescent case of EAS due to a neuroendocrine tumor within a sacrococcygeal teratoma. Complementary PET\/CT on FDG metabolism and SSTR expression is critical for localizing occult neuroendocrine components. Complete tumor resection and multidisciplinary collaboration are key to favorable outcomes, expanding the spectrum of pediatric EAS and providing valuable insight into its evaluation and management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1515\/jpem-2025-0557","type":"journal-article","created":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T19:04:25Z","timestamp":1772305465000},"source":"Crossref","is-referenced-by-count":0,"title":["Challenges of pediatric ectopic ACTH syndrome from sacrococcygeal teratoma"],"prefix":"10.1515","author":[{"given":"Ruochen","family":"Che","sequence":"first","affiliation":[{"name":"Department of Nephrology , Children\u2019s Hospital of Nanjing Medical University , Nanjing , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jianfeng","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery , Children\u2019s Hospital of Nanjing Medical University , Nanjing , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chunlei","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Pathology , Children\u2019s Hospital of Nanjing Medical University , Nanjing , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jingnan","family":"Gao","sequence":"additional","affiliation":[{"name":"Department of Nephrology , Children\u2019s Hospital of Nanjing Medical University , Nanjing , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guoqiang","family":"Shao","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine , Nanjing First Hospital, Nanjing Medical University , Nanjing , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tao","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery , Children\u2019s Hospital of Nanjing Medical University , Nanjing , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-7319-0596","authenticated-orcid":false,"given":"Yuan","family":"Han","sequence":"additional","affiliation":[{"name":"Department of Nephrology , Children\u2019s Hospital of Nanjing Medical University , Nanjing , China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","published-online":{"date-parts":[[2026,2,26]]},"reference":[{"key":"2026022819042183637_j_jpem-2025-0557_ref_001","doi-asserted-by":"crossref","unstructured":"Concepci\u00f3n-Zavaleta, MJ, Armas, CD, Quiroz-Aldave, JE, Garc\u00eda-Villasante, EJ, Gariza-Solano, AC, Durand-V\u00e1squez, MDC, et al.. Cushing disease in pediatrics: an update. Ann Pediatr Endocrinol Metab 2023;28:87\u201397. https:\/\/doi.org\/10.6065\/apem.2346074.037.","DOI":"10.6065\/apem.2346074.037"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_002","doi-asserted-by":"crossref","unstructured":"Mosleh, M, Heo, TG, Shin, HB, Sohn, MJ. Sacrococcygeal teratomas: a case-based review from the perspective of individual multidisciplinary experts. Childs Nerv Syst 2023;39:57\u201364. https:\/\/doi.org\/10.1007\/s00381-022-05768-1.","DOI":"10.1007\/s00381-022-05768-1"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_003","doi-asserted-by":"crossref","unstructured":"Yami Channaiah, C, Karlekar, M, Sarathi, V, Lila, AR, Ravindra, S, Badhe, PV, et al.. Paediatric and adolescent ectopic cushing\u2019s syndrome: systematic review. Eur J Endocrinol 2023;189:S75\u201387. https:\/\/doi.org\/10.1093\/ejendo\/lvad133.","DOI":"10.1093\/ejendo\/lvad133"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_004","unstructured":"Wojcik, M, Kalicka-Kasperczyk, A, Luszawska-Kutrzeba, T, Balwierz, W, Starzyk, JB. The first description of metyrapone use in severe cushing syndrome due to ectopic ACTH secretion in an infant with immature sacrococcygeal teratoma. Case report. Neuro Endocrinol Lett 2015;36:653\u20135."},{"key":"2026022819042183637_j_jpem-2025-0557_ref_005","doi-asserted-by":"crossref","unstructured":"Rydzewska, M, Krawczuk-Rybak, M, Zajkowska, A, Jurczuk, N, Polnik, D, Szalecki, M, et al.. Cushing\u2019s syndrome in infancy due to ectopic ACTH secretion by a sacro-coccygeal teratoma. J\u00a0Pediatr Endocrinol Metab 2017;30:475\u20138. https:\/\/doi.org\/10.1515\/jpem-2016-0339.","DOI":"10.1515\/jpem-2016-0339"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_006","doi-asserted-by":"crossref","unstructured":"Babiker, T, Kyrodimou, E, Berney, DM, Gurnell, M, Drake, WM, Brooke, A. Acromegaly and cushing\u2019s syndrome caused by a neuroendocrine tumor arising within a sacrococcygeal teratoma. Clin Case Rep 2017;5:1768\u201371. https:\/\/doi.org\/10.1002\/ccr3.1148.","DOI":"10.1002\/ccr3.1148"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_007","doi-asserted-by":"crossref","unstructured":"Gadelha, M, Gatto, F, Wildemberg, LE, Fleseriu, M. Cushing\u2019s syndrome. Lancet 2023;402:2237\u201352. https:\/\/doi.org\/10.1016\/S0140-6736(23)01961-X.","DOI":"10.1016\/S0140-6736(23)01961-X"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_008","doi-asserted-by":"crossref","unstructured":"Ahmed, MF, Ahmed, S, Abdussalam, A, Alhusaini, F. A rare case of ectopic adrenocorticotropic hormone syndrome (EAS) in an adolescent girl with a thymic neuroendocrine tumour. Cureus 2024;16:e66615. https:\/\/doi.org\/10.7759\/cureus.66615.","DOI":"10.7759\/cureus.66615"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_009","doi-asserted-by":"crossref","unstructured":"Chioma, L, Patti, G, Cappa, M, Maghnie, M. Cushing syndrome in paediatric population: who and how to screen. J\u00a0Endocrinol Investig 2025;48:7\u201319. https:\/\/doi.org\/10.1007\/s40618-024-02452-w.","DOI":"10.1007\/s40618-024-02452-w"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_010","doi-asserted-by":"crossref","unstructured":"Roberto, G, Aloini, ME, Biondo, I, Ricci, F, Maggio, R, Lardo, P, et al.. Ectopic cushing\u2019s syndrome in pediatric age. Endocr, Metab Immune Disord: Drug Targets 2024;24:26 https:\/\/doi.org\/10.2174\/0118715303333260240715070743.","DOI":"10.2174\/0118715303333260240715070743"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_011","doi-asserted-by":"crossref","unstructured":"Reincke, M, Fleseriu, M. Cushing syndrome: a review. JAMA 2023;330:170\u201381. https:\/\/doi.org\/10.1001\/jama.2023.11305.","DOI":"10.1001\/jama.2023.11305"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_012","unstructured":"Gilbert, R, Lim, EM. The diagnosis of cushing\u2019s syndrome: an endocrine society clinical practice guideline. Clin Biochem Rev 2008;29:103\u20136."},{"key":"2026022819042183637_j_jpem-2025-0557_ref_013","doi-asserted-by":"crossref","unstructured":"Balomenaki, M, Margaritopoulos, D, Vassiliadi, DA, Tsagarakis, S. Diagnostic workup of cushing\u2019s syndrome. J\u00a0Neuroendocrinol 2022;34. https:\/\/doi.org\/10.1111\/jne.13111.","DOI":"10.1111\/jne.13111"},{"key":"2026022819042183637_j_jpem-2025-0557_ref_014","doi-asserted-by":"crossref","unstructured":"Piasecka, M, Papakokkinou, E, Piasecki, A, Falhammar, H, Ragnarsson, O. Survival probabilities in patients with ectopic Cushing\u2019s syndrome-a systematic review and a single-arm meta-analysis. 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