Chirurgische Prävention bei Hereditären
Krebssyndromen

Zusammenfassung

Für die Mehrzahl der klinisch definierten familiären Krebserkrankungen lassen sich mittlerweile molekular definierte hereditäre Krebssyndrome mit bekannten Genmutationen zuordnen. Bei Nachweis der entsprechenden Mutation können die Patienten einem, dem jeweiligen Risikoprofil angepassten, engmaschigen Screening zugeführt werden. Bei bestimmten hereditären Krebssyndromen, wie beispielsweise der FAP und dem hereditären medullären Schilddrüsenkarzinom, ist außerdem bereits die präventive chirurgische Intervention im Sinne einer primären chirurgischen Krebsprävention durch Resektion des gefährdeten Organs etabliert. Bei dem HNPCC-Syndrom und hereditären Magen- und Pankreaskarzinom kann dagegen noch keine allgemein gültige Empfehlung bezüglich eines prophylaktischen Eingriffes aufgestellt werden. Bei der chirurgischen Indikationsstellung gilt es die Penetranz der Karzinomentstehung, weitere mit dem jeweiligen Krebssyndrom assoziierte Erkrankungen und die Größe des durchzuführenden prophylaktischen Eingriffes zu berücksichtigen. Von besonderer Bedeutung ist es, die prophylaktischen Eingriffe mit niedriger Morbidität und Mortalität durchzuführen; es erscheint daher sinnvoll zu sein diese Eingriffe in Zentren mit besonderer Erfahrung zu konzentrieren.

Abstract

The majority of clinically defined familial cancer syndromes have been associated with molecular defined hereditary cancer syndromes with known gene mutations in the recent years. Patients with identified mutations should undergo a screening program adapted to the individual risk profile. Prophylactic surgery with resection of the organ at risk is an accepted option for some of the known cancer syndromes (i. e. FAP, medullary thyroid cancer). For HNPCC and hereditary gastric and pancreatic cancer, however, no recommendations regarding prophylactic surgery can currently be given. Prior to performing a prophylactic resection cancer penetrance, diseases associated with the syndrome and the extent of the surgical procedure have to be considered. It seems to be of high importance to keep the perioperative morbidity and mortality as low as possible in preventive surgery; concentrating prophylactic procedures in centers seems therefore to be a reasonable approach.

Schlüsselwörter

Hereditäre Krebssyndrome - präventive Chirurgie

Key words

Hereditary cancer syndromes - preventive surgery

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