Sindrome di Zollinger-Ellison-Malattie gastrointestinali
Sindrome di Zollinger-Ellison
Che cos’è la sindrome di Zollinger-Ellison?
La sindrome di Zollinger-Ellison è caratterizzata da una triade costituita da:
Ipersecrezione acida gastrica
Ulcerazioni multipla
Tumore delle cellule non β-localizzate nel pancreas o in altri distretti.
La reale incidenza della sindrome di Zollinger-Ellison non è chiara. Da un lato l’ampia diffusione dei potenti farmaci antisecretori ne maschera la sintomatologia, dall’altro la possibile incompletezza del quadro di esordio rende difficoltoso il suo riconoscimento. La prevalenza della sindrome di Zollinger-Ellison sembra non mostrare sostanziali differenze tra maschi e femmine. La lesione responsabile del quadro è rappresentata da un gastrinoma, il più frequente tumore a cellule insulari dopo l’insulinoma. In un terzo dei pazienti con sindrome di Zollinger-Ellison è riconoscibile una sindrome multiendocrina adenomatosa di tipo 1 (MEN-1 gastrinoma, adenoma delle paratiroidi, altri tumori pancreatici).
Manifestazioni cliniche della sindrome di Zollinger-Ellison
La malattia si manifesta con sintomi quali:
Dolore o bruciore epigastrico
Perdita di peso
Senso di debolezza
Ripienezza postprandiale
Nausea e vomito legati alla presenza di ulcerazioni duodenali multiple.
E’ presente diarrea, che nel 30% dei casi precede la comparsa dei sintomi dell’ulcera.
Diagnosi
La diagnosi di ZES è sicura quando, nei pazienti con stomaco intatto, la gastrina plasmatica è superiore a 1000 pg/mL e la secrezione acida basale è superiore a 15 mEq/h; nei pazienti gastrectomizzati, quando la secrezione acida basale è superiore a 5 mmEq/h o vi è una ipergastrinemia con pH inferiore a 2.
Indagini di imaging dell’addome devono essere mirate all’esplorazione delle sedi che costituiscono il cosiddetto “triangolo del gastrinoma”.
L’ecoendoscopia è in grado di rilevare il 50% delle delle localizzazioni duodenali e il 70-90% di quelle pancreatiche.
La scintigrafiacon analoghi radiomarcati della somatostatina, l’ecografia e la TC (Tomografia computerizzata) dell’addome sono indagini meno sensibili, ma permettono di documentare un’ eventuale diffusione metastatica, frequentemente a carico del fegato.
Terapia
La terapia medica si fonda sull’utilizzo di antisecretivi e di farmaci chemioterapici. La somministrazione di octreotide, un analogo sintetico della somatostatina, inibisce il rilascio di gastrina e, di conseguenza, la secrezione acida.
L’asportazione chirurgica della neoplasia, che spesso si avvale dell’endoscopia intraoperatoria, è indicata in assenza di metastasi e ottiene una guarigione nel 40% dei casi. Le recidive sono frequenti e, talvolta, è indicata una gastrectomia totale a scopo palliativo.
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