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Das Nebennierenkarzinom

Diagnostik und Therapie

Adrenocortical carcinoma

Diagnostic work-up and treatment

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Abstract

Das Nebennierenkarzinom (ACC, NN-Karzinom) ist eine seltene Erkrankung mit schlechter Prognose. Präoperativ ist eine sorgfältige Hormondiagnostik obligat, da der Hormonstatus das perioperative Management beeinflusst, und um Tumormarker für die Nachsorge zu etablieren. In der Bildgebung sind CT und MRT gleichwertig, wobei für die Differenzierung zwischen benignen und malignen Raumforderungen die Bestimmung des Fettgehalts der Läsion und das Kontrastmittel-Wash-out nach 10 min von großer Bedeutung sind. Der einzig kurative Ansatz besteht in der kompletten chirurgischen Resektion mittels offener Adrenalektomie (in der Regel über einen lateralen oder thorakoabdominellen Zugang). Eine intraoperative Kapselverletzung ist dabei unbedingt zu vermeiden. Allerdings sind auch nach R0-Resektion Rezidive sehr häufig, sodass regelmäßige Nachsorgeuntersuchungen über mindestens 5 Jahre notwendig sind. Bei fortgeschrittenem Nebennierenkarzinom wird eine Therapie mit Mitotane mit oder ohne Kombination mit einer Chemotherapie, vorzugsweise im Rahmen der FIRM-ACT-Studie, durchgeführt.

Abstract

Adrenocortical carcinoma (ACC) is a rare disease with poor prognosis. Preoperatively, a thorough hormonal work-up is mandatory, as the hormonal status may influence the perioperative management and may also provide marker hormones for monitoring of tumour recurrence. CT and MRI are equally sensitive and specific imaging tools for adrenal tumours. For discerning malignancy, assessment of the fat content of the tumour and contrast media wash-out after 10 min are of great value. Complete surgical resection of the tumour offers the only chance for cure. Open adrenalectomy via a flank or thoracoabdominal approach is the standard surgical technique. Intraoperative tumour spillage should be carefully avoided. Even after R0 resection, recurrence of the disease is frequent and regular follow-up for a minimum of 5 years is required. In advanced ACC, the treatment of choice is mitotane with or without cytotoxic chemotherapy (preferably after inclusion into the FIRM-ACT trial).

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Authors and Affiliations

  1. Schwerpunkt Endokrinologie und Diabetologie, Medizinische Klinik und Poliklinik I, Universitätsklinik, Josef-Schneider-Straße 2, 97080, Würzburg, Deutschland

    S. Johanssen, M. Fassnacht, A.-C. Koschker, S. Hahner & B. Allolio

  2. Urologische Klinik und Poliklinik, Universitätsklinik, Würzburg, Deutschland

    D. Brix & H. Riedmiller

Authors
  1. S. Johanssen
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  2. M. Fassnacht
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  3. D. Brix
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  4. A.-C. Koschker
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  5. S. Hahner
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  6. H. Riedmiller
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  7. B. Allolio
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Correspondence to S. Johanssen.

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Johanssen, S., Fassnacht, M., Brix, D. et al. Das Nebennierenkarzinom. Urologe 47, 172–181 (2008). https://doi.org/10.1007/s00120-007-1578-0

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