Reviews in Endocrine and Metabolic Disorders

, Volume 18, Issue 4, pp 379–380 | Cite as


Neuroendocrine neoplasms
  • Christian A. Koch
  • Stephan Petersenn

It is with great pleasure to introduce Prof. Stephan Petersenn as the guest editor for two special issues on the topic neuroendocrine neoplasms (NEN) in this journal. I have known this top notch expert for the last 15 years after we first had some discussions on how to manage several of such patients with NEN and pursue some research projects. At that time, besides other pursuits, Prof. Petersenn investigated various tissues for the expression of somatostatin receptor subtypes [1, 2]. Prof. Petersenn has brought together a wonderful group of experts in the field NEN and we decided to split the articles into two guest issues of Reviews in Endocrine & Metabolic Disorders.

I myself came across neuroendocrinology and NEN during my internship in neurosurgery. Paraganglioma can even arise in the sellar region [3]. During residency in Internal Medicine at the Ohio State University in Columbus, Ohio, I met Prof. Thomas O’Dorisio who took care of many patients with NEN and collaborated closely with surgeon Prof. Christopher Ellison before moving to the University of Iowa. O’Dorisio’s lab at OSU measured many unique hormone substances including pancreastatin. I reconnected with Prof. O’Dorisio when at Georgetown University encountering a woman with carcinoid syndrome caused by an atypical carcinoid of the uterine cervix [4]. This patient has taught me the “zebra” lesson regarding NEN and the fact that NEN can arise in many tissues. Furthermore, NEN can change their hormonal profile which I have also learned while caring for a woman with Cushing syndrome [5]. NEN can be the reason for secondary forms of diabetes mellitus and a certain form of flushing [6, 7, 8, 9]. Another challenge represents interpreting the measurement of an elevated hormone level and avoiding subsequent costly imaging and other investigations. For instance, serum gastrin can be up to 4000 pg/ml in some patients with pernicious anemia without the presence of a NEN [10]. Given the prevalence of thyroid nodules with the chance, although low, of the NEN medullary thyroid cancer, measuring serum calcitonin is important [11]. Elevated serum calcitonin may be seen with the intake of omeprazole and of fumaric acid, a drug used to treat psoriasis [12, 13, 14]. Establishing correct cutoff and reference ranges for various hormones and deciding when there is “humor” (secretion) from a neuroendocrine tumor remains an ongoing challenge, left alone that there probably are many yet undiscovered hormonal products of NEN [15]. No question, the field of NEN will leave room for more discoveries in the future and we hope you will enjoy this special guest issue in Reviews in Endocrine & Metabolic Disorders.


Compliance with ethical standards

Conflict of interest statement

Christian Koch declares that he has received consulting fees for serving on Advisory Boards of Novartis and declares that he has no conflict of interest related to this article.


  1. 1.
    Unger N, Serdiuk I, Sheu SY, Walz MK, Schulz S, Schmid KW, et al. Immunohistochemical determination of somatostatin receptor subtypes 1,2A, 3,4, and 5 in various adrenal tumors. Endocr Res. 2004;30(4):931–4. Scholar
  2. 2.
    Unger N, Ueberberg B, Schulz S, Saeger W, Mann K, Petersenn S. Differential expression of somatostatin receptor subtype 1-5 proteins in numerous human normal tissues. Exp Clin Endocrinol Diabetes. 2012;120(8):482–9. Scholar
  3. 3.
    Osamura RY, Kovacs K, Lopes MBS, Mete O, Nishioka H. Paraganglioma. Page 50. In: Lloyd RV, Osamura RY, Kloppel G, Rosai J, editors. WHO classification of Tumours of endocrine organs, 4th edition, volume 10. Lyon: WHO Blue Book Series; 2017. ISBN: 978-92-832-4493-6.Google Scholar
  4. 4.
    Koch CA, Azumi N, Furlong M, Jha RC, Kehoe T, Trowbridge C, et al. Carcinoid syndrome caused by an atypical carcinoid of the uterine cervix. J Clin Endocrinol Metab. 1999;84(11):4209–13. Scholar
  5. 5.
    Miehle K, Tannapfel A, Lamesch P, Borte G, Schenker E, Kluge R, et al. Pancreatic neuroendocrine tumor with ectopic ACTH-production upon second recurrence. J Clin Endocrinol Metab. 2004;89(8):3731–6. Scholar
  6. 6.
    East HE, Subauste JS, Gandhi A, Koch CA. About secondary causes of diabetes mellitus. J Miss State Med Assoc. 2012;53(11):380–3.PubMedGoogle Scholar
  7. 7.
    Trantakis C, Koch CA, Tannapfel A, Meixensberger J, Drynda K, Führer D. Acromegaly caused by a thoracic neuroendocrine tumor. Exp Clin Endocrinol Diabetes. 2005;S67:113–58.Google Scholar
  8. 8.
    Florez JC, Shepard JA, Kradin RL. Case records of the Massachusetts General Hospital. Case 17-2013. A 56-year-old woman with poorly controlled diabetes mellitus and fatigue. N Engl J Med. 2013;368(22):2126–36. Scholar
  9. 9.
    Hannah-Shmouni F, Stratakis CA, Koch CA. Flushing in (neuro)endocrinology. Rev Endocr Metab Disord. 2016;17(3):373–80. Scholar
  10. 10.
    Lewin KJ, Dowling F, Wright JP, Taylor KB. Gastric morphology and serum gastrin levels in pernicious anemia. Gut. 1976;17(7):551–60. Scholar
  11. 11.
    Kahaly GJ, Algeciras-Schimnich A, Davis TE, Diana T, Feldkamp J, Karger S, et al. United States and European multicenter prospective study for the analytical performance and clinical validation of a novel sensitive fully automated immunoassay for calcitonin. Clin Chem. 2017;63(9):1489–96. Scholar
  12. 12.
    Vitale G, Ciccarelli A, Caraglia M, Galderisi M, Rossi R, Del Prete S, et al. Of two provocative tests for calcitonin in medullary thyroid carcinoma: omeprazole vs. pentagastrin. Clin Chem. 2002;48(9):1505–10.PubMedGoogle Scholar
  13. 13.
    Erdoğan MF, Güllü S, Başkal N, Uysal AR, Kamel N, Erdoğan G. Omeprazole: calcitonin stimulation test for the diagnosis follow-up and family screening in medullary thyroid carcinoma. J Clin Endocrinol Metab. 1997;82(3):897–9. Scholar
  14. 14.
    Kuroda K, Inhibitory AM. Effect of fumaric acid and dicarboxylic acids on gastric ulceration in rats. Arch Int Pharmacodyn Ther. 1977;226(2):324–30.PubMedGoogle Scholar
  15. 15.
    Allelein S, Ehler M, Morneau C, Schwartz K, Goretzki PE, Seppel T, et al. Measurement of basal serum calcitonin for the diagnosis of medullary thyroid cancer. Horm Metab Res 2018;50(1):23–28.Google Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Medicover Oldenburg MVZOldenburgGermany
  2. 2.Department of Medicine IIITechnical University of DresdenDresdenGermany
  3. 3.University of LouisvilleLouisvilleUSA
  4. 4.ENDOC CenterHamburgGermany

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