Abstract
Background
Malignant pheochromocytomas are rare tumors comprising about 15% of all pheochromocytomas. A specific diagnosis of malignancy requires evidence of local invasion or metastases. The purpose of this paper is to review current management of pheochromocytomas proven to be malignant.
Methods
Both a review of the literature and case studies of more than 100 patients with proven malignant pheochromocytoma seen at the University of Michigan Hospitals from 1980 to 1992 are the basis of management recommendations. Representative individual cases are used to illustrate treatment principles.
Results
Malignancy as evidence by local invasion, particularly peritumor veins, does not preclude complete resection. Bone is the most common site of distant metastases. The131I-MIBG scintiscan is the most useful means of identifying occult metastases.
Conclusions
Aggressive surgical resection of local recurrences and soft tissue metastases can result in excellent palliation. Adrenergic blockade is a cornerstone of treatment, regardless whether surgical resection, triple drug therapy,131I-MIBG or external radiation therapy is ultimately utilized.
Zusammenfassung
Grundlagen
Maligne Phäochromozytome sind seltene Tumoren. Rund 15% aller Phäochromozytome sind bösartig. Lokal invasives Wachstum oder Metastasen sind Hinweise auf Malignität. In vorliegender Übersicht soll das zur Zeit gängige Vorgehen bei malignen Phäochromozytomen dargestellt werden.
Methodik
Anhand einer Literaturübersicht und anhand von Kasuistiken der Jahre 1980 bis 1992, ausgewähtl aus mehr als 100 Patienten mit nachgewiesenen, an der Universität von Michigan behandelten, malignen Phäochromozytomen, werden Therapieeempfehlungen abgeleitet. Besonders eindrucksvolle Krankheitsverläufe wurden zur besonderen Darstellung der Behandlungsprinzipien herangezogen.
Ergebnisse
Malignität, gekennzeichnet durch lokales, invasives Wachstum besonders in große Venen, schließt radikale Resektionen nicht aus. Fernmetastasen sind vorwiegend in Knochen lokalisiert. Okkulte Metastasen können mit dem131J-MIBG-Scan nachgewiesen werden.
Schlußfolgerungen
Eine aggressive, chirurgische Resektion von Lokalrezidiven und Weichteilmetastasen bewirkt eine optimale Palliation. Eine adrenerge Blockade ist die Grundlage für jede Therapie, egal ob chirurgisch, medikamentös, mit131J-MIBG oder durch externe Strahlentherapie.
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Thompson, N.W. Malignant pheochromocytoma. Acta Chir Austriaca 25, 235–239 (1993). https://doi.org/10.1007/BF02602112
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DOI: https://doi.org/10.1007/BF02602112