Pankreatischer Hyperinsulinismus – Wandel des Krankheitsbildes mit spezifischen Unterschieden auch bei sporadischen Erkrankungsformen (Eigene Erfahrung an 144 operierten Patienten von 1986–2009)

Zusammenfassung

Der pankreatogene Hyperinsulinismus wurde in früheren Jahren fast synonym mit einem gutartigen oder bösartigen Insulinom gleich gesetzt und nur die familiären Formen z. B. im Rahmen des MEN-1-Syndroms wurden getrennt davon betrachtet. So konzentrierte sich die chirurgische Therapie fast ausschließlich auf die Technik der Operation, mit dem Vergleich offener und minimal-invasiver Verfahrensweisen. Mit Diagnose von Patienten, die an einem sporadischen Hyperinsulinismus ohne Insulinom (non-insulinoma pancreatogenic hypoglycemia syndrome = NIPHS) leiden, wurde nun klinisch wie auch pathologisch (Insulinomatose) eine zusätzliche Gruppe von Patienten definiert, die das bisherige diagnostische und operative Vorgehen erweitert hat. Anhand der eigenen Erfahrung an 144 operierten Patienten der letzten 22 Jahre mit 16 Patienten, die an NIPHS litten, und anhand der Literatur wird dargestellt, welche Voraussetzungen heute an die Chirurgie des pankreatogenen Hyperinsulinismus gestellt werden müssen.

Abstract

The diagnoses of pancreatogenic hyperinsulinism and insulinoma (benign or malignant) were almost synonomously used during the last decades. Only familial forms of hyperinsulinism, i. e., in patients with multiple endocrine neoplasia type 1 were separately discussed. The surgical literature concentrated on technical questions, comparing open and minimal invasive techniques. The clinical diagnosis of patients with pancreatogenic hypo­glycaemia syndrome (NIPHS) and the pathological diagnosis of insulinomatosis has now opened up new questions in the diagnosis and therapy of pancreatogenic hyperinsulinism. On the basis of our experience from 144 patients oper­ated on for pancreatogenic hyperinsulinism during the last 22 years with 16 NIPHS patients and with the help of the relevant literature, we explain the prerequisites that surgical therapy has to fulfil in the treatment of patients with pancreatogenic hyperinsulinism today.

endokrine Chirurgie - pankreatischer Hyperinsulinismus - neuroendokrine Tumoren - Insulinom - NIPHS

endocrine surgery - pancreatic hyperinsulininsm - neuroendocrine tumours - insulinoma - NIPHS

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