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Ektope Hormonsekretion bei neuroendokrinen Tumoren

Ectopic hormone secretion by neuroendocrine tumors

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Zusammenfassung

Die ektope Hormonproduktion bei neuroendokrinen Tumoren ist eine seltene Komplikation. Am häufigsten werden Tumoren beobachtet, die das Kortikotropin-Releasing-Hormon (CRH) oder adrenokortikotrope Hormon (ACTH) produzieren, wodurch klassische Zeichen eines Cushing-Syndroms hervorgerufen werden können. Daneben finden sich Tumoren, die das zur Akromegalie führende „growth hormone releasing hormon“ (GHRH) freisetzen oder eine inadäquate Sekretion des antidiuretischen Hormons (ADH) aufweisen, die typischerweise eine Hyponatriämie auslöst. Andere paraneoplastische Hormonsekretionszustände wie die Gonadotropinsekretion werden selten beobachtet. Üblicherweise lässt sich bei ektoper Hormonproduktion kein hypophysärer Tumor nachweisen. Die Diagnosestellung erfolgt über die Messung der zirkulierenden Peptide, die oft durch bildgebende Verfahren, beispielsweise die Somatostatinrezeptorszintigraphie, unterstützt wird. Primär ist die Therapie auf eine kurative Behandlung des Tumorleidens ausgerichtet. Bei fortgeschrittenen Tumoren wird durch die palliative Therapie mit antihormonell aktiven Substanzen wie den Somatostatinanaloga eine Symptomkontrolle angestrebt.

Abstract

Ectopic hormone production is a rare complication in neuroendocrine tumors. Tumors producing corticotropin-releasing hormone (CRH) and adrenocorticotropic hormone (ACTH) are most commonly observed, leading to the classical symptoms of Cushing’s syndrome. Additionally, a very low percentage of neuroendocrine tumors can produce growth hormone-releasing hormone (GHRH) leading to classical features of acromegaly. Moreover, ectopic antidiuretic hormone (ADH) secretion has been described in neuroendocrine tumors presenting as hyponatremia due to the syndrome of inappropriate ADH secretion. Other ectopic hormone secretions, such as paraneoplastic gonadotropin release are rarely observed. Ectopic hormone secretion is not usually associated with a detectable pituitary mass and diagnosis is based on the measurement of circulating peptides. This is frequently assisted by imaging techniques, such as somatostatin receptor scintigraphy. Therapeutically a curative approach is the primary goal but in advanced tumors palliative treatment aims to control symptoms with the help of specific antihormonal compounds, such as somatostatin analogues.

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Authors and Affiliations

  1. Medizinische Klinik I, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland

    C. Hubold

  2. Experimentelle und Klinische Endokrinologie, Medizinische Klinik I, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Lübeck, Deutschland

    G. Brabant

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Correspondence to C. Hubold.

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Die Autoren C. Hubold und G. Brabant haben zu gleichen Teilen zu dieser Arbeit beigetragen.

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Hubold, C., Brabant, G. Ektope Hormonsekretion bei neuroendokrinen Tumoren. Internist 53, 145–151 (2012). https://doi.org/10.1007/s00108-011-2920-6

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