Diagnostik und operative Therapie von Phäochromozytomen und Paragangliomen

Zusammenfassung

Phäochromozytome und Paragangliome sind seltene chromaffine Tumoren, welche aufgrund ­einer begleitenden Katecholamin-Produktion eine besondere Herausforderung für den Chirurgen darstellen. Neue Erkenntnisse zur Genese insbesondere hereditärer Tumoren haben die alte Regel des 10 %-Tumors relativiert und die Anforderungen an die notwendige Diagnostik sowie die operative Strategie wesentlich verändert. Die frühzeitige Erkennung eines malignen Prozesses oder synchroner hereditärer Tumoren sind Ziel verschiedener bildgebender Verfahren wie CT / MRT, ¹²³J-MIBG-(SPECT) oder ¹⁸F-DOPA-PET. Ohne Vorliegen von Metastasen ist eine sichere Einschätzung der Dignität präoperativ und sogar histopathologisch selten eindeutig möglich. Wesentlic­he Voraussetzung für eine risikoarme Operation ist die adäquate präoperative α-Blockade, welche üblicherweise auf 3–5 mg / kg KG / Tag langsam gesteigert werden sollte. Einen Sonderfall stellen ausschließlich dopaminsezernierende Para­gang­lio­me dar. Therapie der Wahl ist für singuläre ­benigne Phäochromozytome die minimalinvasive Resektion, welche retroperitoneoskopisch oder transabdominell erfolgen kann. Auch abdomi­nelle Paragangliome können überwiegend minimalinvasiv entfernt werden; sie erfordern in der Regel jedoch eine besondere Expertise. Für sehr große oder malignitätsverdächtige Tumoren sowie ggf. bei multifokalen Prozessen haben sich transabdominelle Resektionen bewährt. Auch bei fortgeschrittenen malignen Prozessen sind Debulking-Resektionen unter palliativen Aspekten sinnvoll und tragen zur Verbesserung der Lebensqualität und der Prognose der Patienten bei.

Abstract

Pheochromocytomas and paragangliomas are rare chromaffin tumours that represent an exceptional challenge for the surgeon because of the concomitant secretion of catecholamines. Recent findings on the genetic background of hereditary tumours have challenged the rule of the 10 % ­tumour and significantly changed the requirements for preoperative work-up and surgical strat­egy. Early detection of malignant growth or multiple hereditary tumours is the goal of imag­ing techniques such as CT / MRI, ¹²³I-MIBG-(SPECT) or ¹⁸F-DOPA-PET. However, in the absence of metastasis, reliable differentiation between ­benign and malignant growth is preoperatively and even histopathologically rarely possible. An essential precondition for successful surgical ther­apy with low operative risks is an ade­quate pretreatment with α-adrenergic antag­o­nists which should slowly be increased to 3–5 mg / kg BW / day prior to resection. Dopamine-secreting paragangliomas represent the sole exception. Minimally invasive techniques using a transabdominal or retroperitoneal approach have become the gold standard for the resection of unifocal benign pheochromocytomas. In addition, most paragangliomas located below the dia­phragm can be resected with a minimally invasive approach which, however, demands exceptional expertise. Open transabdominal resections are an approved therapy for large or potentially ­malignant tumours and for settings with multi­focal tumour sites. Even for advanced malignant tumours, surgical debulking may be reasonable to improve the patient’s quality of life and prognosis.

Schlüsselwörter

Phäochromozytom - Paragangliom - MEN - PGL - endokrine Chirurgie - Viszeralchirurgie

Key words

pheochromocytoma - paraganglioma - MEN - PGL - endocrine surgery - abdominal surgery

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