{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T12:13:35Z","timestamp":1789215215829,"version":"build-2803163510"},"reference-count":35,"publisher":"The Endocrine Society","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2004,8,1]]},"abstract":"<jats:p>We present a 54-yr-old woman with ectopic corticotropin syndrome caused by a neuroendocrine tumor of the pancreas. At initial presentation, the patient suffered from diarrhea, heartburn, and nonspecific abdominal pain. There was no evidence of Cushing\u2019s syndrome. A neuroendocrine tumor in the head of the pancreas with metastases into peripancreatic lymph nodes was diagnosed and completely resected. Fourteen months later, abdominal computed tomography and scintigraphy with 111In-labeled octreotide suggested relapse of the tumor. The patient again had no evidence of Cushing\u2019s syndrome. A second in toto tumor resection was performed. Another 8 months later, the patient developed forgetfulness, depressive episodes, muscle weakness, new-onset hypertension, hypokalemia, plethora, diabetes mellitus, polyuria, and weight loss. Endocrine testing suggested a source of ectopic ACTH production. An octreotide scan showed an intense uptake ventromedial of the left kidney, an area that showed a mass lateral of the superior mesenteric artery on abdominal magnetic resonance imaging. A complete pancreatectomy with splenectomy and left-sided adrenalectomy were performed. At this second relapse, this neuroendocrine tumor clinically had changed its hormonal profile. Immunohistochemically, in contrast to primary tumor and first relapse, we found strong immunostaining for ACTH in tumor cells of the second relapse and a MIB-1 index greater than 20%. To our knowledge, this is the first report describing a pancreatic neuroendocrine tumor that started to secrete ACTH de novo at the time of the second relapse after two former complete tumor resections. This case underscores the pluripotency of neuroendocrine tumor cells and the importance of keeping in mind a possible shift in hormone production during tumor evolution and progression.<\/jats:p>","DOI":"10.1210\/jc.2003-032164","type":"journal-article","created":{"date-parts":[[2004,8,3]],"date-time":"2004-08-03T21:40:43Z","timestamp":1091569243000},"page":"3731-3736","source":"Crossref","is-referenced-by-count":29,"title":["Pancreatic Neuroendocrine Tumor with Ectopic Adrenocorticotropin Production upon Second Recurrence"],"prefix":"10.1210","volume":"89","author":[{"given":"Konstanze","family":"Miehle","sequence":"first","affiliation":[{"name":"Departments of Endocrinology and Nephrology (K.M., R.P., C.A.K.), 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Tannapfel","sequence":"additional","affiliation":[{"name":"Pathology (A.T.), 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Lamesch","sequence":"additional","affiliation":[{"name":"Surgery (P.L., R.A.O., W.K.), 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gudrun","family":"Borte","sequence":"additional","affiliation":[{"name":"Radiology (G.B.), 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eva","family":"Schenker","sequence":"additional","affiliation":[{"name":"Gastroenterology, Hematology, and Oncology (E.S.), 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Regine","family":"Kluge","sequence":"additional","affiliation":[{"name":"Nuclear Medicine (R.K.), University of Leipzig, 04103 Leipzig, Germany; Volker Wiechmann: 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rudolf A.","family":"Ott","sequence":"additional","affiliation":[{"name":"Surgery (P.L., R.A.O., W.K.), 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Volker","family":"Wiechmann","sequence":"additional","affiliation":[{"name":"Department of Pathology, St. Georg Hospital (V.W.), 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Koch","sequence":"additional","affiliation":[{"name":"Department of Pathology, Charite, Humboldt University of Berlin (M.K.), 10117 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Woubet","family":"Kassahun","sequence":"additional","affiliation":[{"name":"Surgery (P.L., R.A.O., W.K.), 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ralf","family":"Paschke","sequence":"additional","affiliation":[{"name":"Departments of Endocrinology and Nephrology (K.M., R.P., C.A.K.), 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian A.","family":"Koch","sequence":"additional","affiliation":[{"name":"Departments of Endocrinology and Nephrology (K.M., R.P., C.A.K.), 04129 Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","reference":[{"issue":"Suppl 2","key":"2020071619245298100_R1","doi-asserted-by":"crossref","first-page":"S111","DOI":"10.1093\/annonc\/12.suppl_2.S111","article-title":"Chemotherapy and biotherapy in the treatment of neuroendocrine tumours","volume":"12","author":"Oberg","year":"2001","journal-title":"Ann Oncol"},{"key":"2020071619245298100_R2","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1159\/000063164","article-title":"Neuroendocrine tumors of the gastro-entero-pancreatic system: the role of early diagnosis, genetic testing and preventive surgery.","volume":"20","author":"Plockinger","year":"2002","journal-title":"Dig Dis"},{"key":"2020071619245298100_R3","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1007\/s00268-001-0017-4","article-title":"Nonsurgical treatment of advanced malignant neuroendocrine pancreatic tumors and midgut carcinoids.","volume":"25","author":"Skogseid","year":"2001","journal-title":"World J Surg"},{"key":"2020071619245298100_R4","first-page":"283","article-title":"Clinical and laboratory studies of ectopic humoral syndromes.","volume":"25","author":"Liddle","year":"1969","journal-title":"Recent Prog Horm Res"},{"key":"2020071619245298100_R5","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1677\/erc.0.0100459","article-title":"The importance of the measurement of circulating markers in patients with neuroendocrine tumours of the pancreas and gut.","volume":"10","author":"Ardill","year":"2003","journal-title":"Endocr Rel Cancer"},{"key":"2020071619245298100_R6","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/S0046-8177(75)80097-9","article-title":"Multiple hormone producing islet cell carcinomas of the pancreas. 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