Familiäre Pankreatitis und assoziiertes Pankreaskarzinom
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Zusammenfassung
Bei etwa 4% der Patienten mit chronischer Pankreatitis besteht eine Familienanamnese. Die Mehrzahl dieser Patienten erkrankt erstmals im Kindes- und Jugendalter; bei immerhin ca. 35% liegt das Manifestationsalter jenseits des 21. Lebensjahres. Charakteristisch ist eine langsame Progression der Erkrankung im Vergleich zur chronisch-alkoholischen Pankreatitis. Nach den Literaturdaten besteht ein 40%iges Lebenszeitrisiko, an einem Pankreaskarzinom zu erkranken, obwohl die Lebenserwartung des Gesamtkollektiv nicht verminderst sein soll. Es existiert kein bildgebendes Verfahren, mit dem man das Malignom in der chronisch entzündeten Drüse zuverlässig nachweisen kann. Folglich ist der Stellenwert von invasiven Screeningverfahren (MRT, Endosonographie etc.) nicht belegt und man ist hier auf klassische klinische Kriterien angewiesen. Bei Rauchern tritt das Pankreaskarzinom deutlich häufiger bzw. früher auf, so dass den Patienten dringend zur Abstinenz geraten werden sollte.
Schlüsselwörter
Hereditäre Pankreatitis Diabetes mellitus Pankreaskarzinom Prophylaxe RauchenFamilial pancreatitis and associated pancreatic carcinoma
Abstract
Familial pancreatitis is found in 4% of patients with chronic pancreatitis. In the majority, the onset is in childhood or adolescence. Approximately 35% report experiencing their first symptoms when older than 21 years. Typically, the disease progress (characterized by duct dilatation, calcification, and diabetes) is slow compared with alcoholic chronic pancreatitis. In the literature, a 40% lifetime risk for pancreatic cancer has been reported for these patients, although overall survival for this group is not different than for controls. No imaging technique reliably detects pancreatic cancer in patients with chronic pancreatitis; therefore, screening strategies may be questioned at this time. Pancreatic cancer develops earlier and more frequently in smokers than in nonsmokers, so patients with familial pancreatitis should be emphatically advised not to smoke.
Keywords
Hereditary pancreatitis Diabetes Pancreatic carcinoma Prophylaxis SmokingNotes
Interessenkonflikt
Der Autor gibt an, dass kein Interessenkonflikt besteht.
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