Gastrinoma Tanı ve Tedavi Yaklaşımları
Özet
Gastrinoma, insülinomadan sonra en sık karşılaşılan fonksiyonel pankreatik nöroendokrin tümör olup, çoğunlukla duodenum ve pankreasta yerleşir. Aşırı gastrin salgılanmasıyla karakterize olan bu hastalık, refrakter peptik ülserler, şiddetli epigastrik ağrı ve steatore tarzında diyare ile kendini gösteren Zollinger-Ellison Sendromu (ZES) tablosuna yol açar. Vakaların yaklaşık yüzde yirmi beşi kalıtımsal MEN1 sendromu ile ilişkilidir ve bu durum malignite potansiyeli taşıyan tümörlerin daha genç yaşlarda, küçük boyutlu ve çok odaklı ortaya çıkmasına neden olur. Tanı aşamasında, en az bir hafta önce proton pompa inhibitörleri kesilerek açlık serum gastrin düzeyinin ölçülmesi ve gastrik pH değerinin ikinin altında olduğunun saptanması kritik öneme sahiptir; şüpheli durumlarda sekretin provokasyon testi uygulanır. Tümörün lokalizasyonunda ve metastazların tespitinde günümüzde EUS, somatostatin reseptör sintigrafisi ve Ga-68 PET/BT öne çıkmaktadır. Tedavinin temel amacı sporadik vakalarda cerrahi rezeksiyon ve bölgesel lenf nodu diseksiyonu ile tam kür sağlamaktır. Ameliyat esnasında İOUS, duodenotomi ve palpasyon teknikleri tümörün yerini belirlemede etkin olarak kullanılır. Medikal tedavide ise gastrik asit hipersekresyonunu ve buna bağlı gelişebilecek perforasyon, kanama gibi komplikasyonları kontrol altına almak amacıyla proton pompa inhibitörleri ilk seçenek olarak uzun süreli güvenle uygulanmaktadır.
Gastrinoma is the second most common functional pancreatic neuroendocrine tumor after insulinoma, predominantly localized within the duodenum and pancreas. Characterized by autonomous hypersecretion of gastrin, it induces Zollinger-Ellison Syndrome (ZES), which manifests with refractory peptic ulcers, severe epigastric pain, and steatorrhea-type diarrhea. Approximately twenty-five percent of cases are linked to the hereditary MEN1 syndrome, causing these potentially malignant tumors to present at a younger age as small, multicentric lesions. For accurate diagnosis, fasting serum gastrin levels and a gastric pH below two must be demonstrated at least one week after discontinuing proton pump inhibitors; secretin stimulation testing is utilized in borderline cases. For localization and staging of metastases, EUS, somatostatin receptor scintigraphy, and Ga-68 PET/CT are currently the preferred modalities. The primary goal of treatment in sporadic cases is to achieve a complete cure via surgical resection combined with regional lymphadenectomy. Intraoperative palpation, IOUS, and duodenotomy are effectively employed to precisely locate the tumors during surgery. In terms of medical management, long-term administration of proton pump inhibitors remains the gold standard to control gastric acid hypersecretion and prevent life-threatening ulcer complications such as perforation or hemorrhage.
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