Black swans - neuroendocrine tumors of rare locations

  • Christian A. KochEmail author
  • S. Petersenn


Neuroendocrine neoplasms (NEN) are rare and heterogeneous. Therefore, they often remain unrecognized for many years, causing significant disease burden. We here report on four unusual NEN presentations including a metastatic NEN of the kidney, hypoglycemia caused by an insulin-like growth factor-2-oma (previously called non-islet-cell tumor hypoglycemia), multifocal pheochromocytoma in von Hippel Lindau syndrome, and ileal NEN metastatic to the heart. One could say that each one of these tumors were “black swans” and learning about them will increase further awareness of the spectrum of NEN.


Neuroendocrine tumor Kidney Carcinoid Heart von Hippel Lindau Pheochromocytoma Hypoglycemia Insulin-like growth factor 2 


Compliance with ethical standards

Conflict of interest

Prof. Koch declares no direct conflict with this article. He has served on the Advisory Board of Novartis on the topic acromegaly and has participated in educational conferences on the topics neuroendocrine tumors and acromegaly sponsored by Novartis and Ipsen. Prof. Petersenn declares no direct conflict with this article. He has served on the Advisory Board of Ipsen on the topics of acromegaly and NEN.

Informed consent

Patients provided informed consent for publication.


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Authors and Affiliations

  1. 1.Medicover GmbHBerlin / HannoverGermany
  2. 2.Carl von Ossietzky University of OldenburgOldenburgGermany
  3. 3.Technical University of DresdenDresdenGermany
  4. 4.University of LouisvilleLouisvilleUSA
  5. 5.ENDOC Center for Endocrine TumorsHamburgGermany

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