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Endokrine paraneoplastische Syndrome

  • N. Reisch
  • M. Reincke
Originalien
  • 7 Downloads

Zusammenfassung

Endokrine paraneoplastische Syndrome sind die Folge der Sekretion bioaktiver Substanzen aus neoplastischen Zellen endokrinen oder neuroendokrinen Ursprungs, die in einer Vielzahl von Organen vorkommen. Typischerweise sind sie in der Lunge, dem Gastrointestinaltrakt, dem Pankreas, der Schilddrüse, dem Nebennierenmark, der Haut, Prostata oder Brust lokalisiert. Charakteristisch für endokrine paraneoplastische Syndrome ist, dass die Sekretion von Peptiden, Aminen oder anderen bioaktiven Substanzen ektop ist und die Symptome nicht durch den physiologischen Ursprung bedingt sind. Die klinische Präsentation aufgrund einer ektopen Hormonsekretion ist jedoch nicht zu unterscheiden von einem zunächst erwarteten eutopen endokrinen Tumor, was eine diagnostische Herausforderung darstellt. Am häufigsten werden Tumoren mit einer Sekretion von antidiuretischem Hormon (Folge: Hyponatriämie), von adrenokortikotropem Hormon bzw. sehr selten Kortikotropin-Releasing-Hormon (Folge: Cushing-Syndrom) oder von Growth-Hormone-Releasing-Hormon (Folge: Akromegalie) beobachtet. Die meisten endokrinen paraneoplastischen Syndrome nichtendokriner Tumoren kommen bei hochmalignen Tumoren vor. Sie korrelieren aber nicht immer mit Tumorstadium, malignem Potenzial oder Prognose. Da die ektope Hormonproduktion eine seltene Komplikation ist, gibt es kaum evidenzbasierte Therapieempfehlungen. Im Vordergrund steht die Therapie der Grunderkrankung, im Falle einer palliativen Therapie kann symptomatisch therapiert werden.

Schlüsselwörter

Syndrom der inadäquaten Sekretion von antidiuretischem Hormon Cushing-Syndrom Akromegalie Ektope Hormone Positronenemissionstomographie Computertomographie 

Endocrine paraneoplastic syndromes

Abstract

Endocrine paraneoplastic syndromes result from the production of bioactive substances from neoplastic cells, of endocrine or neuroendocrine origin. Typically these are located in the lungs, the gastrointestinal tract, pancreas, thyroid gland, adrenal medulla, skin, prostate or breast. In endocrine paraneoplastic syndromes the secretion of peptides, amines or other bioactive substances is always ectopic and not related to the anatomical source. The clinical presentation, however, is indistinguishable from a suspected eutopic endocrine tumor posing a diagnostic challenge. The most common endocrine paraneoplastic syndromes are based on the secretion of antidiuretic hormone (ADH) resulting in hyponatremia, secretion of adrenocorticotropic hormone (ACTH) or rarely corticotropin-releasing hormone (CRH) resulting in Cushing syndrome as well as secretion of growth hormone-releasing hormone resulting in acromegaly. Paraneoplastic endocrine syndromes mainly occur in highly malignant tumors; however, the development of these tumors does not necessarily correlate with tumor stage, malignant potential or prognosis. As endocrine paraneoplastic syndromes are a rare complication, there are hardly any evidence-based therapeutic recommendations. Treatment of the underlying tumor is the first choice and in a palliative setting symptomatic therapy is possible.

Keywords

Inappropriate ADH syndrome Cushing syndrome Acromegaly Hormones, ectopic Positron emission tomography computed tomography 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

N. Reisch und M. Reincke geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Copyright information

© Springer-Verlag GmbH Austria, ein Teil von Springer Nature 2018

Authors and Affiliations

  1. 1.Medizinische Klinik IVKlinikum der Universität MünchenMünchenDeutschland

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