Sindrome di Zollinger-Ellison: cause, sintomi e cura

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La Sindrome di Zollinger-Ellison è una condizione complessa in cui uno o più tumori (gastrinomi) si formano nel pancreas o nella parte superiore del piccolo intestino (duodeno): secernono grandi quantità di gastrina un ormone che stimola la produzione di acido gastrico che a sua volta porta ad ulcere peptiche. E’ una malattia rara che può verificarsi in qualsiasi momento della vita, ma è più frequente tra i 30 ed i 50 anni. Scopriamo quali sono le cause, i sintomi e le possibilità di cura.

Sindrome Zollinger -Ellison

Sintomi

I sintomi della sindrome di Zollinger-Ellison possono includere:

  • Dolore addominale
  • Diarrea
  • Bruciore, dolore o disagio nella parte superiore dell’addome
  • Reflusso acido e bruciore di stomaco
  • Nausea e vomito
  • Debolezza
  • Sanguinamento nel tratto digestivo
  • Perdita di peso non intenzionale
  • Diminuzione dell’appetito
  • Anemia

E’ importante consultare il medico se si hanno questi sintomi, specie se persistenti, avvisandolo se si è fatto uso di farmaci da banco contro l’acidità di stomaco per lungo tempo (come l’omeprazolo, cimetidina, famotidina o la ranitidina) . Questi farmaci possono infatti mascherare i sintomi, e ritardare la diagnosi. Se si è affetti di sindrome di Zollinger-Ellison, la diagnosi precoce e il trattamento sono importanti.

Cause

La causa esatta della sindrome di Zollinger-Ellison è attualmente sconosciuta. Ma la sequenza di eventi che si verifica è chiara. La sindrome inizia quando un tumore (gastrinoma) o tumori si formano nel pancreas, duodeno o nei linfonodi adiacenti al pancreas.

Il pancreas si trova dietro e sotto lo stomaco. Produce enzimi che sono essenziali per la digestione del cibo, oltre che la gastrina, un ormone che controlla la produzione di acido gastrico. Oltre a causare la produzione di acido in eccesso, i gastrinomi possono essere cancerogeni (maligni). I tumori stessi crescono lentamente, ma possono diffondersi altrove – più comunemente ai linfonodi vicini o al fegato.

La Sindrome di Zollinger-Ellison può essere provocata da una malattia ereditaria chiamata neoplasia endocrina multipla di tipo I (MEN I).

Anche se i sintomi possono richiedere il medico di famiglia, è probabile che questi indirizzi ad uno specialista gastroenterologo o oncologo.

Diagnosi

Il medico dovrà basare una diagnosi sui seguenti punti:

 

    • Storia medica. Fondamentale sarà stilare una anamnesi precisa: avere familiarità con la MEN I rende più probabile la sindrome di Zollinger-Ellison.
    • Gli esami del sangue. Un campione di sangue viene analizzato per valutare i livelli di gastrina: è un test che si fa a digiuno e per il quale occorrerebbe sospendere l’assunzione di farmaci antiacidi.
    • Endoscopia del tratto gastrointestinale superiore. Serve per valutare la presenza di ulcere e fare una biopsia alla ricerca di un gastrinoma.
    • Studi di imaging: TAC, ecografia o risonanza magnetica.

Trattamenti e farmaci

Nel trattamento della sindrome di Zollinger-Ellison, i medici curano i tumori e le ulcere. Se il medico può rimuovere il tumore, la cura delle ulcere potrebbe non essere necessaria. Un’operazione per rimuovere i tumori che si verificano in Zollinger-Ellison richiede un chirurgo esperto perché in genere si tratta di masse piccole e difficili da individuare. La resezione del tumore però non è l’unica opzione e non sempre è quella possibili: può essere necessaria l’asportazione di parte del fegato, l’embolizzazione o l’ablazione con radiofrequenza. Ma anche chemioterapia o trapianto di fegato. La chirurgia può essere utile anche per trattare un’ulcera perforata.

La produzione di acido in eccesso può quasi sempre essere controllata. I farmaci noti come inibitori della pompa protonica sono la prima linea di trattamento e sono i comuni antiacidi:

  • lansoprazolo
  • omeprazolo
  • pantoprazolo
  • rabeprazolo
  • esomeprazolo

L’uso prolungato però di questi medicinali è associato ad un aumentato rischio di fratture dell’anca, del polso e della colonna vertebrale, secondo la Food and Drug Administration. Questo rischio va valutato con i benefici.

In alternativa si possono usare gli antagonisti dei recettori H2 o:

  • Ranitidina
  • Nizatidina
  • Famotidina
  • Cimetidina

Fonte: Mayo Clinic

  

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