Indikation und operative Verfahren bei MEN1-assoziierten duodenopankreatischen neuroendokrinen Neoplasien

Zusammenfassung

Die optimale Therapie von duodenopankreatischen neuroendokrinen Neoplasien (dpNEN), die im Rahmen der multiplen endokrinen Neoplasie Typ 1 auftreten, stellt nach wie vor eine große Herausforderung dar und wird teilweise kontrovers diskutiert. Aufgrund der Rarität der Erkrankung fehlen prospektiv randomisierte Studien, sodass die meisten Empfehlungen bez. der Operationsindikation und des -verfahrens auf retrospektiven Fallserien basieren. Prinzipiell ist eine operative Therapie bei nicht funktionellen dpNEN > 2 cm, bei Malignitätsverdacht und bei funktionell aktiven dpNEN indiziert. Hierbei kommen eine Enukleation oder formale Pankreasresektionen mit oder ohne Lymphadenektomie in Betracht. Ziel der Therapie sollte es sein, hormonassoziierte Symptome zu beseitigen und einem aggressiven metastasierenden Verlauf vorzubeugen. Zugleich sollte bei den zumeist jungen Patienten die Pankreasfunktion und Lebensqualität durch möglichst parenchymsparende Resektionen erhalten werden.

Abstract

The optimal therapy of duodenopancreatic neuroendocrine neoplasia (dpNEN), which occurs in the context of multiple endocrine neoplasia type 1, is still a major challenge and is controversial. Due to the rarity of the disease, there is a lack of prospective randomised studies, so that most recommendations regarding the surgical indication and procedure are based on retrospective case series. In summary, surgical therapy is indicated for non-functional dpNEN > 2 cm, suspected malignancy and functionally active dpNEN. Enucleation or formal pancreatic resections with or without lymphadenectomy may be considered. The aim of therapy should be to eliminate hormone-associated symptoms and prevent an aggressive metastatic disease. At the same time, pancreatic function and quality of life should be preserved in the mostly young patients by resections that save as much parenchyma as possible.

Schlüsselwörter

endokrine Chirurgie - pankreatische neuroendokrine Neoplasien - Multiple endokrine Neoplasie Typ 1 - Insulinom - Gastrinom - nicht funktionelle pankreatische neuroendokrine Neoplasien

Keywords

endocrine surgery - multiple endocrine Neoplasie Type 1 - insulinoma - gastrinoma - duodenopancreatic neuroendocrine neoplasia

Publication History

Received: 15 March 2023

Accepted after revision: 28 May 2023

Article published online:
21 August 2023

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