Apendiks Vermiformis Tümörleri
Özet
Apendiks vermiformis tümörleri, oldukça heterojen yapıya sahip ve farklı malign potansiyeller barındıran nadir neoplazmlardır. En sık görülen histopatolojik alt tipleri nöroendokrin tümörler (NET), müsinöz neoplazmalar, goblet hücreli adenokarsinomlar, kolonik tip adenokarsinomlar ve taşlı yüzük hücreli adenokarsinomlardır. Bu tümörler genellikle klinik olarak akut apandisiti taklit ettikleri için çoğunlukla operasyon sonrası histopatolojik inceleme ile teşhis edilirler. Tanı ve evreleme süreçlerinde kromogranin A ve serotonin gibi biyobelirteçlerin yanı sıra bilgisayarlı tomografi, MRG ve somatostatin reseptör görüntülemesi sağlayan Ga68-DOTATATE PET/BT gibi modern görüntüleme yöntemlerinden yararlanılır. Tümörlerin tedavi algoritması histopatolojik dereceye, tümör boyutuna ve yayılım durumuna göre değişir; düşük riskli ve küçük lezyonlarda tek başına apendektomi yeterli olurken, büyük veya agresif tümörlerde sağ hemikolektomi standardı oluşturur. Peritoneal metastaz gelişen seçilmiş vakalarda sitoredüktif cerrahi ve HIPEC kombinasyonu sağkalımı anlamlı ölçüde artırırken, ileri evre ve yüksek dereceli hastalıklarda kolorektal kanser protokollerine benzer florourasil bazlı perioperatif ve adjuvan sistemik kemoterapi rejimleri tercih edilmektedir.
Appendiceal neoplasms represent a heterogeneous group of rare tumors that exhibit diverse malignant potentials and complex clinical behaviors. The primary histopathological subtypes include neuroendocrine tumors (NETs), mucinous neoplasms, goblet cell adenocarcinomas, colonic-type adenocarcinomas, and aggressive signet-ring cell adenocarcinomas. Because these lesions frequently mimic the clinical presentation of acute appendicitis, the definitive diagnosis is predominantly established postoperatively through histopathological evaluation of appendectomy specimens. Diagnostic and staging pathways utilize tumor markers like chromogranin A and serotonin, complemented by contrast-enhanced CT, MRI, and advanced somatostatin receptor imaging via Ga68-DOTATATE PET/CT. Treatment management is highly dictated by tumor size, grade, and nodal involvement; simple appendectomy suffices for small, well-differentiated tumors, whereas right hemicolectomy is mandatory for larger or high-risk invasive malignancies. In cases with peritoneal carcinomatosis, cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC) significantly improves survival rates. Furthermore, advanced or high-grade diseases necessitate systemic fluorouracil-based chemotherapeutic regimens, mirroring established colorectal cancer treatment algorithms to optimize oncological outcomes.
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