Adrenal Kortikal Tümörler
Özet
Adrenal kortikal tümörler, benign karakterdeki adrenal kortikal adenomlar (AKA) ve malign özellikteki adrenal kortikal karsinomlar (AKK) olmak üzere iki ana grupta incelenmektedir. En sık saptanan benign neoplazi olan AKA’lar, genellikle nonfonksiyonel olup insidental olarak saptanırken; fonksiyonel olanları Cushing sendromu veya hiperaldosteronizme yol açabilmektedir. Makroskopik olarak homojen, sarı renkli ve genellikle 5 cm’den küçük izlenen AKA’larda nekroz ve invazyon saptanmazken, mikroskopik düzeyde lipitten zengin berrak veya kompakt hücrelerin karışımı gözlenir. Buna karşılık nadir görülen malign AKK’lar, kadınlarda ve sol adrenal bezde daha sık izlenmekte, genellikle 4 cm’den büyük, düzensiz sınırlı, kesit yüzeyinde nekroz ve kanama barındıran heterojen kitleler olarak prezente olmaktadır. AKK’larda yuvalı büyüme paterni yerini solid ve trabeküler yapılara bırakırken; kapsül, vasküler ve sinüzoidal invazyonlar ile mm² başına artmış mitoz (>5) ve yüksek Ki67 proliferasyon indeksi (>%5) malignite tanısında ve evrelemesinde kritik rol oynamaktadır. Ayırıcı tanıda ve karsinom ayrımında Weiss skorlama sistemi, retikülin algoritması ile Lin-Weiss-Bisceglia ve Wieneke gibi spesifik kriterlerin yanı sıra, kortikal kökeni doğrulamada en güvenilir biyobelirteç olan SF-1 panelinden ve karsinomlarda paranükleer pozitiflik veren IGF2 immünhistokimyasal ekspresyonundan yararlanılmaktadır.
Adrenal cortical tumors are primarily classified into two main groups: benign adrenal cortical adenomas (ACA) and malignant adrenal cortical carcinomas (ACC). ACAs, the most frequently encountered benign neoplasms, are generally nonfunctional and discovered incidentally, whereas functional variants can lead to Cushing's syndrome or hyperaldosteronism. Macroscopically observed as homogeneous, yellow, and usually smaller than 5 cm, ACAs exhibit no necrosis or invasion, while microscopically showing a mixture of lipid-rich clear or compact cells. Conversely, rare and malignant ACCs are more common in females and the left adrenal gland, typically presenting as irregular masses larger than 4 cm with heterogeneous cross-sections containing extensive necrosis and hemorrhage. In ACCs, the nested growth pattern is replaced by solid and trabecular structures; furthermore, capsular, vascular, and sinusoidal invasions, along with an increased mitotic count per mm² (>5) and a high Ki67 proliferation index (>%5), play a critical role in malignancy diagnosis and grading. For differential diagnosis and distinguishing carcinomas, scoring models like the Weiss system, reticulin algorithm, and specific Lin-Weiss-Bisceglia or Wieneke criteria are utilized, alongside the SF-1 panel, which is the most reliable biomarker for confirming cortical origin, and the immunohistochemical expression of IGF2, which shows paranuclear positivity exclusively in carcinomas.
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