This Article Full Text Full Text (PDF) Submit a related Letter to the Editor Purchase Article View Shopping Cart Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Miehle, K. Articles by Koch, C. A. Search for Related Content PubMed PubMed Citation Articles by Miehle, K. Articles by Koch, C. A. Pubmed/NCBI databases Substance via MeSH Medline Plus Health Information Pancreatic Cancer

Pancreatic Neuroendocrine Tumor with Ectopic Adrenocorticotropin Production upon Second Recurrence

Departments of Endocrinology and Nephrology (K.M., R.P., C.A.K.), Pathology (A.T.), Surgery (P.L., R.A.O., W.K.), Radiology (G.B.), Gastroenterology, Hematology, and Oncology (E.S.), and Nuclear Medicine (R.K.), University of Leipzig, 04103 Leipzig, Germany; Volker Wiechmann: Department of Pathology, St. Georg Hospital (V.W.), 04129 Leipzig, Germany; and Department of Pathology, Charite, Humboldt University of Berlin (M.K.), 10117 Berlin, Germany

Address all correspondence and requests for reprints to: Christian A. Koch, MD, FACP, FACE, Department of Endocrinology and Nephrology, University of Leipzig, Philipp Rosenthalstrasse 27, 04103 Leipzig, Germany. E-mail: kocc{at}medizin.uni-leipzig.de.

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’s 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’s 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.

Abbreviations: CA, Cancer antigen; CT, computed tomography; MRI, magnetic resonance imaging; MTC, medullary thyroid carcinoma; PRL, prolactin.

This article has been cited by other articles:

				C. A KOCH, O. GIMM, A. O VORTMEYER, H. K AL-ALI, P. LAMESCH, R. OTT, R. KLUGE, U. BIERBACH, and A. TANNAPFEL Does the Expression of c-kit (CD117) in Neuroendocrine Tumors Represent a Target for Therapy? Ann. N.Y. Acad. Sci., August 1, 2006; 1073: 517 - 526. [Abstract] [Full Text] [PDF]

				R. L Brown, T. Muzzafar, R. Wollman, and R. E Weiss A pituitary carcinoma secreting TSH and prolactin: a non-secreting adenoma gone awry. Eur. J. Endocrinol., May 1, 2006; 154(5): 639 - 643. [Abstract] [Full Text] [PDF]