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Isolated primary aldosteronism in adrenocortical carcinoma: A case report and review of literature

Isolierter primärer Aldosteronismus bei Nebennierenkarzinom: Kasuistik und Literaturübersicht

  • Main Topics: Adrenal Tumors
  • Adrenocortical Tumors
  • Published:
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Abstract

Background

Adrenocortical carcinoma synthesizing solely mineralocorticoids is an unusual disorder which has only been reported in literature in 22 other patients.

Methods

This case report describes a patient presenting with hypokalaemia, hypertension, metabolic acidosis and a left adrenal tumor. Extensive laboratory testing allowed the diagnosis of pure primary hyperaldosteronism without secretion of other steroids.

Results

The treatment of choice after establishing the diagnosis biochemically is to remove the tumor surgically. Histologically the lesion within the left adrenal was classified as a benign adrenocortical adenoma. After recurrence of, disease 6 months postoperatively, a widespread retroperitoneal tumor was en bloc resected and malignancy was confirmed by the histological features. When extended total histological examination of the first tumor was performed, capsular invasion was detected in one slice and the diagnosis of adrenocortical cancer was established retrospectively not only on the basis of clinical behavior. Despite precise surgical treatment of the recurrence and chemotherapy, the patient died 14 months after the diagnosis of primary hyperaldosteronism.

Conclusions

Close biochemical and clinical follow-up examinations are necessary in each patient with hormonlly active tumors. Sometimes only the postoperative course discriminates benign and malignant lesions.

Zusammenfassung

Grundlagen

Adrenokortikale Karzinome mit alleiniger Sekretion von Mineralokortikoiden sind sehr selten. In der Literatur finden sich nur 22 gut dokumentierte Fälle.

Methodik

Diese Kasuistik beschreibt einen Patienten mit Hypokaliämie, Hypertonie, metabolischer Azidose und mit einem linksseitigen Nebennierentumor. Die genaue laborchemische Untersuchung erbrachte die ausschließliche Sekretion von Aldosteron.

Ergebnisse

Nach Sicherung der biochemischen Diagnose erfolgte als Behandlung der Wahl die chirurgische Entfernung des Tumors. Die histologische Untersuchung der Nebenniere erbrachte zunächst ein gutartiges Nebennierenrindenadenom. 6 Monate später wurde klinisch und biochemisch ein Tumorrezidiv beobachtet. Es erfolgte en bloc die Exstirpation eines großen, malignen, retroperitonealen Tumors. Nun erfolgte retrospektive die neuerliche histologische Aufarbeitung des ersten Präparats. In einem Schnitt fand sich eine Kapselinvasion als Zeichen eines adrenokortikalen Karzinoms. Trotz erweiterter chirurgischer Resektion und Chemotherapie starb der Patient 14 Monate nach Erstdiagnose des priären Hyperaldosteronismus.

Schlußfolgerungen

Engmaschige biochemische und klinische postoperative Untersuchungen sind bei allen endokrinen Tumoren durchzuführen. Vielfach ermöglicht erst der postoperative Verlauf die Unterscheidung zwischen gut- und bösartigen Tumoren.

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Authors and Affiliations

  1. Department of Internal Medicine III Division of Clinical Endocrinology, University of Vienna, Vienna

    B. Ludvik & Maria Templ

  2. Department of Surgery I, University of Vienna, Medical School, Spitalgasse 2, A-1090, Vienna

    B. Niederle, R. Roka & F. Längle

  3. Department of Pathology, University of Vienna, Vienna

    N. Neuhold

  4. Department of Internal Medicine I, Krankenanstalt Rudolfstiftung, Vienna

    G. Schernthaner

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Ludvik, B., Niederle, B., Roka, R. et al. Isolated primary aldosteronism in adrenocortical carcinoma: A case report and review of literature. Acta Chir Austriaca 25, 212–216 (1993). https://doi.org/10.1007/BF02602106

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