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Testikuläre adrenale Resttumoren im Hoden bei Adoleszenten

Testicular adrenal rest tumors in adolescents

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Zusammenfassung

Hintergrund

Testikuläre adrenale Resttumoren (TART) treten häufig bei Patienten mit klassischem adrenogenitalem Syndrom (AGS) auf und können im Kindesalter sowie in der Adoleszenz erkannt und therapiert werden. Die Detektion von TART ist aufgrund der häufig schwierigen Abgrenzung zu anderen testikulären Raumforderungen problematisch.

Methode

Eine umfassende endokrinologische und sonographische Untersuchung ist zwingend erforderlich. Wenngleich es sich um benigne Läsionen handelt, so ist eine Behandlung mit hochdosierten Glukokortikoiden und/oder Mineralokortikoiden v. a. zur Erhaltung oder zum Wiedererlangen der Fertilität sinnvoll. In Abhängigkeit des Stadiums der Erkrankung sowie des Ansprechens auf die medikamentöse Therapie ist eine operative Resektion der Befunde zu erwägen.

Ergebnisse

Durch eine konsequente Behandlung und ein engmaschiges Therapiemonitoring können Langzeitfolgen in den meisten Fällen vermieden werden. Derzeit existiert kein validiertes einheitliches Therapiekonzept, was durch die Heterogenität der Krankheitsbilder und Verläufe bzw. durch die unklare Datenlage begründet ist, so dass diese in spezialisierten Zentren erfolgen sollte.

Abstract

Background

Testicular adrenal rest tumors (TART) frequently occur in patients with congenital adrenal hyperplasia (CAH) and can be detected and treated in childhood as well as in adolescence. Due to the intricate dilimitation to other testicular masses the correct diagnosis of TART can be problematic. An extensive endocrinologic evaluation and ultrasound examination are mandatory. Even though TART are benign lesions a high-dose therapy with glucocorticoid and/or mineralocorticoid suppletion is necessary for protecion or regain of fertility.

Methods

A surgical approach can be considered, depending on stage of disease and response on drug therapy. Consequent treatment and constant therapy monitoring might significantly improve long-term outcome.

Results

Currently there is no validated standard therapy concept, which can be explained by the heterogenity of disease patterns progression and the limited data available, respectively. Therefore treatment should be subject to specialized centres.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. P. Hüppe und I. Rübben geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Sektion für Andrologie, Klinik für Urologie, Universitätsklinikum Essen, Hufelandstraße 55, 45122, Essen, Deutschland

    P. Hüppe

  2. Sektion für Kinderurologie, Klinik für Urologie, Universitätsklinikum Essen, Essen, Deutschland

    I. Rübben

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  1. P. Hüppe
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Correspondence to P. Hüppe.

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Hüppe, P., Rübben, I. Testikuläre adrenale Resttumoren im Hoden bei Adoleszenten. Urologe 53, 196–200 (2014). https://doi.org/10.1007/s00120-013-3386-z

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