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Das Phäochromozytom – noch immer eine Herausforderung

Pheochromocytoma – still a challenge

  • Schwerpunkt: Therapierefraktäre und sekundäre arterielle Hypertonie
  • Published:
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Zusammenfassung

Das Phäochromozytom ist ein seltener, in der Regel gutartiger, katecholaminproduzierender Tumor des Nebennierenmarks oder der Paraganglien. Die tumorbedingte Hormonwirkung führt zur Leitsymptomatik mit Palpitationen, Schweißattacken und Kopfschmerzen sowie arterieller Hypertonie. Biochemisch wird die Diagnose durch die Bestimmung der Katecholamine und fraktionierten Metanephrine im 24-h-Sammelurin oder der freien Metanephrine im Plasma gesichert. Als bildgebende Verfahren werden die Computertomographie und Magnetresonanztomographie der Nebennieren eingesetzt, komplementär die 123Jod-MIBG-Szintigraphie und 18Fluor-Dopa-Positronenemissionstomographie. Da etwa 24% der scheinbar sporadischen Phäochromozytome familiären Ursprungs sind, ist die molekulargenetische Untersuchung unverzichtbarer Bestandteil der Diagnostik. Die endoskopische und organerhaltende Chirurgie ist nach ausreichender präoperativer α-Rezeptorblockade als kurativer Ansatz die Therapie der Wahl. Aufgrund des Rezidiv- und Malignitätsrisikos sind regelmäßige Nachsorgeuntersuchungen unverzichtbar.

Abstract

Pheochromocytomas are rare, mostly benign catecholamine-producing tumors arising from the chromaffin cells of the adrenal medulla or in the paraganglia. Clinical presentation is highly variable but typically with hypertension, severe headaches, palpitations and sweating. Biochemical testing by 24 h urinary fractioned metanephprines or catecholamines and plasma free metanephrines as the most sensitive screening approach, confirms the catecholamine excess. Computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen as well as functional imaging with 123Iod-MIBG scintigraphy and 18F-dopa positron emission tomography are used for tumor localization. Because approximately a quarter of tumors develop secondary to germ-line mutations, screening for genetic alterations is important. The therapy of choice is the endoscopic adrenal sparing surgery following preoperative α-blockade. Regular follow-up remains essential due to possible recurrence and malignancy.

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Authors and Affiliations

  1. Medizinische Klinik – Innenstadt, Klinikum der Universität München, München, Deutschland

    N. Reisch

  2. Endocrinolgy and Metabolism, Institute of Biomedical Research, University of Birmingham, Birmingham, Großbritannien

    N. Reisch

  3. Klinik für Chirurgie und Zentrum für Minimal Invasive Chirurgie, Kliniken Essen-Mitte, Essen, Deutschland

    M.K. Walz

  4. Abteilung Innere Medizin IV, Medizinische Universitätsklinik, Universitätsklinikum Freiburg, Hugstetter Straße 55, 79106, Freiburg i. Br., Deutschland

    Z. Erlic &  H.P.H. Neumann

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Reisch, N., Walz, M., Erlic, Z. et al. Das Phäochromozytom – noch immer eine Herausforderung. Internist 50, 27–35 (2009). https://doi.org/10.1007/s00108-008-2196-7

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  • DOI: https://doi.org/10.1007/s00108-008-2196-7

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