Pankreasın Nöroendokrin Tümörleri
Özet
Pankreas nöroendokrin tümörleri (PNET), langerhans adacık hücrelerinden kaynaklanan ve nadir görülen heterojen bir tümör grubudur. Son yıllarda görüntüleme tekniklerinin gelişmesiyle insidansı artan bu tümörler, hormon salgılama durumlarına göre fonksiyonel ve non-fonksiyonel olarak ikiye ayrılır. Fonksiyonel tümörler ürettikleri hormonlara göre insülinoma, gastrinoma, VİPoma, glukagonoma ve somatostatinoma olarak adlandırılır ve spesifik klinik tablolara yol açar. Tanılamada immünohistokimyasal belirteçler (Kromogranin A, Sinaptofizin) ve çok fazlı kontrastlı BT, MRG, EUS ile Galyum-68 PET-BT gibi gelişmiş görüntüleme yöntemleri kritik rol oynar. PNET'lerin sınıflandırılmasında ve evrelemesinde DSÖ'nün Ki-67 proliferasyon indeksi ile AJCC sistemleri esas alınmaktadır. Tedavi stratejileri tümörün boyutuna, evresine ve işlevselliğine göre değişmekte olup; küçük, asemptomatik non-fonksiyonel tümörler için takip önerilirken, büyük veya semptomatik lezyonlarda tek küratif seçenek cerrahi rezeksiyondur. Rezeke edilemeyen ya da metastatik vakalarda ise somatostatin analogları, hedefe yönelik tedaviler ve kemoterapi gibi sistemik seçenekler devreye girer. Sonuç olarak, PNET'lerin yönetimi onkolog, radyolog, endokrinolog ve patologlardan oluşan multidisipliner bir ekip çalışması gerektirmektedir.
Pancreatic neuroendocrine tumors (PNETs) are rare, heterogeneous neoplasms arising from the Langerhans islet cells. Characterized by an increasing incidence due to advanced cross-sectional imaging, PNETs are broadly categorized into functional and non-functional tumors. Functional PNETs, which hypersecrete specific hormones, cause distinct clinical syndromes and include insulinomas, gastrinomas, VIPomas, glucagonomas, and somatostatinomas. Definitive diagnosis relies on immunohistochemical markers such as Chromogranin A and Synaptophysin, alongside advanced imaging modalities including multiphasic CT, MRI, EUS, and Gallium-68 DOTATATE PET-CT. Grading and staging are dictated by the WHO classification based on the Ki-67 proliferation index and the AJCC staging manuals. Management strategies depend heavily on tumor size, function, and stage; localized active surveillance is considered safe for asymptomatic tumors smaller than 2 cm, whereas surgical resection remains the only curative standard for symptomatic or larger tumors. For unresectable or metastatic disease, systemic therapies including somatostatin analogs, targeted pharmacological agents, and cytotoxic chemotherapy are utilized to stabilize tumor growth and mitigate symptoms. Ultimately, optimal PNET management mandates a highly collaborative, multidisciplinary team approach involving surgical oncologists, medical oncologists, radiologists, endocrinologists, and pathologists.
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