Somatostatinoma Tanı ve Tedavi Yaklaşımları
Özet
Somatostatinomalar, aşırı miktarda somatostatin içeren ve bazen salgılayan D hücre kökenli, yıllık insidansı 40 milyonda 1 olan oldukça nadir nöroendokrin tümörlerdir. Ortalama tanı yaşı 50-55 arası olup cinsiyet dağılımı eşittir. Tümörlerin yaklaşık %55'i pankreasta, kalanı ise duodenum, jejunum gibi gastrointestinal bölgelerde yerleşir. Olguların çoğunluğu (%75) malign karakterdedir ve metastaz ile başvurur. Klinik olarak safra kesesi kontraktilitesinde azalmaya bağlı kolelitiazis, azalan insülin sekresyonu nedeniyle diabetes mellitus/glukoz intoleransı, pankreatik enzim inhibisyonu sonucu diyare ve steatore triadından oluşan "somatostatinoma sendromu" görülür. Tanıda açlık plazma somatostatin düzeyinin 30 pg/mL'yi aşması önemlidir. Tümör lokalizasyonunda BT, MRG, endoskopik ultrasonografi ve özellikle yüksek duyarlılığa sahip Ga-68 DOTATOC/DOTATATE PET/BT tercih edilir. Tedavide ilk seçenek cerrahi rezeksiyondur (pankreatikoduodenektomi). İlerlemiş veya cerrahiye uygun olmayan metastatik olgularda semptom kontrolü ve tümör büyümesini baskılamak amacıyla somatostatin analogları (oktreotid, lanreotid), hepatik arter embolizasyonu, ablasyon tedavileri, everolimus gibi hedefe yönelik moleküler ajanlar ve peptit reseptörü radyoligand tedavileri uygulanır. Metastatik hastalık varlığında prognoz genellikle kötüdür.
Somatostatinomas are rare neuroendocrine tumors originating from D cells that contain and occasionally secrete excessive amounts of somatostatin, with an annual incidence of 1 in 40 million. The average age of diagnosis is between 50 and 55 years, showing an equal gender distribution. Approximately 55% of these tumors are located in the pancreas, while the remaining arise in gastrointestinal sites such as the duodenum or jejunum. The majority (75%) are malignant and present with metastatic disease. Clinically, they present with the "somatostatinoma syndrome" triad: cholelithiasis due to inhibited gallbladder contractility, diabetes mellitus or glucose intolerance from decreased insulin secretion, and diarrhea/steatorrhea resulting from pancreatic enzyme inhibition. Diagnosis is confirmed when fasting plasma somatostatin levels exceed 30 pg/mL. For tumor localization, multiphase CT, MRI, endoscopic ultrasound, and highly sensitive functional imaging methods like Ga-68 DOTATOC/DOTATATE PET/CT are utilized. Surgical resection (pancreaticoduodenectomy) is the preferred treatment modality. For advanced or unresectable metastatic cases, somatostatin analogs (octreotide, lanreotide) are used as first-line therapy to control symptoms, alongside hepatic artery embolization, ablation, molecular targeted agents like everolimus, and peptide receptor radionuclide therapy. When metastatic disease is present at diagnosis, the overall prognosis remains poor.
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