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Neue Standards in der Diagnostik und Therapie des Nebennierenkarzinoms

New standards in the diagnostics and therapy of adrenocortical carcinoma

  • Leitthema
  • Published:
Der Onkologe Aims and scope

Zusammenfassung

Hintergrund

Das Nebennierenkarzinom ist eine seltene endokrine Tumorerkrankung, deren Pathogenese noch nicht vollständig verstanden ist.

Ziel

Ziel des Beitrags ist eine kompakte Darstellung der aktuellen Diagnostik und Therapie des Nebennierenkarzinoms.

Material und Methode

Selektive Literaturrecherche.

Ergebnisse

Leitsymptome resultieren aus Steroidhormonexzess und der lokalen Raumforderung. Zunehmend erfolgt die Diagnosestellung zufällig im Rahmen einer aus anderen Gründen durchgeführten Bildgebung des Abdomens. Fortschritte in Diagnostik und Therapie konnten innerhalb weniger Jahre durch internationale Zusammenarbeit erzielt werden. Die Herangehensweise bei Verdacht auf ein Nebennierenkarzinom ist grundsätzlich interdisziplinär. Der klinische Verdacht wird durch eine umfassende Hormonanalytik und eine standardisierte Schnittbildgebung erhärtet. Eine chirurgische R0-Resektion ist das primäre Therapieziel. Die meisten Patienten profitieren von einer adjuvanten Therapie mit Mitotan. Bei metastasierter Erkrankung ist die Therapie der ersten Wahl Mitotan alleine oder in Kombination mit Etoposid, Doxorubicin und Cisplatin.

Schlussfolgerung

Aufgrund der Seltenheit der Erkrankung ist bei Verdacht auf ein Nebennierenkarzinom eine Kontaktaufnahme mit einer spezialisierten Einrichtung ratsam.

Abstract

Context

Adrenocortical carcinoma is a rare endocrine malignancy, the pathogenesis of which is only partially understood.

Objective

The aim of this article is to provide a concise description of the current diagnostic and therapeutic procedures in adrenocortical carcinoma.

Materials and methods

A selective review of the literature was carried out.

Results

The clinical suspicion of adrenocortical carcinoma is indicated by the presence of symptoms related to steroid hormone excess and abdominal mass effects. Tumors are increasingly discovered incidentally on cross-sectional imaging and the differentiation from the much more common adrenal adenoma is crucial. The approach to patients with adrenocortical carcinomas is multidisciplinary and the diagnosis is made by comprehensive laboratory work-up and standardized abdominal imaging. Oncological resection is the primary goal of treatment. After surgery adjuvant treatment with mitotane is indicated in the majority of patients. In metastatic disease mitotane alone or in combination with etoposide, doxorubicine and cisplatin is the treatment of first choice.

Conclusions

Suspicion of adrenocortical carcinoma should trigger consultation with a specialized referral center for this disease.

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Authors and Affiliations

  1. Medizinische Klinik und Poliklinik I, Schwerpunkt Endokrinologie und Diabetologie, Universitätsklinikum Würzburg, Oberdürrbacher Straße 6, 97080, Würzburg, Deutschland

    T. Deutschbein &  M. Kroiss

  2. Chirurgische Klinik und Poliklinik I, Universitätsklinikum, Universität Würzburg, Würzburg, Deutschland

    J. Reibetanz

  3. Klinik für Nuklearmedizin, Klinikum Augsburg, Augsburg, Deutschland

    M.C. Kreissl

  4. Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, München, Deutschland

    M. Fassnacht

Authors
  1. T. Deutschbein
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  2. J. Reibetanz
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  3. M.C. Kreissl
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  4. M. Fassnacht
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  5. M. Kroiss
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Corresponding author

Correspondence to M. Kroiss.

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Deutschbein, T., Reibetanz, J., Kreissl, M. et al. Neue Standards in der Diagnostik und Therapie des Nebennierenkarzinoms. Onkologe 19, 200–208 (2013). https://doi.org/10.1007/s00761-012-2390-z

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  • DOI: https://doi.org/10.1007/s00761-012-2390-z

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