Pankreatik Nöroendokrin Tümörler
Özet
Pankreatik nöroendokrin tümörler (pNET), pankreastaki nöroendokrin hücrelerden köken alan ve tüm pankreas neoplazmlarının yaklaşık %1-2’sini oluşturan nadir tümörlerdir. Bu tümörler, hormon salgılayıp semptom vermelerine göre fonksiyone ve non-fonksiyone olarak iki ana gruba ayrılır; non-fonksiyone tümörler genellikle daha sinsi seyredip ileri evrede teşhis edilirken, fonksiyone pNET'ler (insülinoma, gastrinoma, glukagonoma gibi) spesifik klinik tablolarla ortaya çıkar. Teşhis ve takip süreçlerinde kromogranin A ve NSE gibi genel biyobelirteçlerin yanı sıra hormona özgü belirteçler ve ileri görüntüleme yöntemleri (BT, MRI, EUS) kritik rol oynar. Evreleme sistemleri zaman içinde evrilerek pNET'lerin adenokarsinomlardan farklı biyolojik doğasını yansıtacak şekilde AJCC ve ENETS kriterlerine göre güncellenmiştir. DSÖ sınıflaması ise tümörlerin diferansiyasyon derecesini ve Ki-67 proliferasyon indeksini baz alarak NET ve NEC ayrımı yaparak prognoz tayinine olanak tanır. Tedavi ve sağkalım başarısı, tümörün evresi, tipi ve metastaz durumuyla doğrudan ilişkilidir.
Pancreatic neuroendocrine tumors (pNETs) are rare neoplasms originating from neuroendocrine cells, accounting for approximately 1-2% of all pancreatic tumors, and are classified into functional and non-functional types based on hormone secretion and clinical symptoms. While non-functional pNETs are often diagnosed at advanced stages due to their silent nature, functional pNETs such as insulinomas, gastrinomas, and glucagonomas present with specific clinical syndromes, requiring general biomarkers like chromogranin A and NSE alongside specialized imaging techniques like CT, MRI, and EUS for diagnosis. Staging and classification systems have evolved through AJCC and ENETS guidelines to distinguish the unique biological behavior of pNETs from adenocarcinomas, with the WHO classification utilizing the Ki-67 index and tumor differentiation to categorize them into neuroendocrine tumors (NETs) or carcinomas (NECs). Ultimately, the prognosis and management of these tumors depend heavily on their histopathological characteristics, stage at diagnosis, and the presence of metastases, necessitating a comprehensive diagnostic approach for optimal patient outcomes.
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