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Clinical presentation, biochemical parameters and localization of catecholamine-secreting tumors

Klinisches Bild, biochemische Parameter und Lokalisation katecholaminproduzierender Tumoren

  • Main Topics: Adrenal Tumors
  • Pheochromocytomas
  • Published:
Acta Chirurgica Austriaca Aims and scope Submit manuscript

Abstract

Background

The diagnosis of these catecholamine-secreting tumors requires clinical awareness of the various presentations and syndromes in which these tumors are found. Any suspicion of a pheochromocytoma warrants aggressive diagnostic intervention. The aim of the present paper is to describe clinical manifestations, biochemical work-up and localization of pheochromocytoma.

Methods

Basic diagnostic method is urinary catecholamines and/or their metabolites. For confirmation, plasma catecholamines, other neuropeptides, and clonidine suppression test can be helpful in confirming the diagnosis.

Results

Once the diagnosis of pheochromocytomas has been established, alpha receptor blockade should be instituted prior to further investigations. Localization of the tumor is essential prior to surgical intervention. CT or MRI scanning are the first line modalities for localization of these tumors. If CT or MRI scan fails to localize the tumor, the patient has recurrent/malignant disease, or bilateral disease is suspected, then I-131 or I-123 MIBG scanning should be performed.

Conclusions

Pheochromocytoma are potentially lethal but usually benign tumors. High degree of clinical suspicion and accurate work-up is essential for successful outcome.

Zusammenfassung

Grundlagen

Grundlage für die Diagnose katecholaminproduzierender Tumoren ist die Kenntnis der verschiedensten klinischen Präsentationen und Syndrome. Jeder Verdacht auf ein Phäochromozytom zwingt zur aggressiven diagnostischen Abklärung. Ziel vorliegender Zusammenstellung ist das Beschreiben der klinischen Manifestation, der biochemischen Abklärung und der Lokalisation.

Methodik

Die Bestimmung von Harnkatecholaminen und ihrer Metaboliten bilden die diagnostische Basis. Zur Bestätigung der Verdachtsdiagnose dient die Bestimmung von Plasmakatecholaminen und anderer Neuropeptide. Weiters kann der Clonidin-Suppressionstest hilfreich sein.

Ergebnisse

Sobald die Diagnose eines Phäochromozytoms feststeht, sollte noch vor weiteren Untersuchungen mit einer α-Rezeptorblockade begonnen werden. Die Lokalisation des Tumors ist vor jedem chirurgischen Vorgehen notwending. CT oder MRI sind die ersten lokalisationsdia-gnostischen Maßnahmen. Sollte CT oder MRI den Tumor nicht lokalisieren können, liegt ein Rezidiv oder eine Malignität vor oder besteht der Verdacht auf eine bilaterale Erkrankung, sollte ein Jod-131- oder Jod-123-MIBG-Scan durchgeführt werden.

Schlußfolgerungen

Phäochromozytome sind eventuell tödlich, überwiegend aber gutartig. Bei klinischem Verdacht bildet eine sorgfältige Untersuchung die Grundlage für eine erfolgreiche Behandlung.

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Authors and Affiliations

  1. Department of Surgery, Karolinska Hospital, S-104 01, Stockholm, Sweden

    S. Gröndal & B. Hamberger M.D.

  2. University of Calgary, Alberta, Canada

    Janice L. Pasieka

Authors
  1. Janice L. Pasieka
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  2. S. Gröndal
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  3. B. Hamberger M.D.
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Additional information

Supported by The Detweiler Clinical Traineeship, Royal College of Physicians and Surgeons of Canada, the Swedish Medical Research Council (2330) and the Karolinska Institute.

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Pasieka, J.L., Gröndal, S. & Hamberger, B. Clinical presentation, biochemical parameters and localization of catecholamine-secreting tumors. Acta Chir Austriaca 25, 220–223 (1993). https://doi.org/10.1007/BF02602108

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  • DOI: https://doi.org/10.1007/BF02602108

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