Adrenal Bezin Nadir Görülen Primer Neoplazileri
Özet
Böbrek üstü bezinin (adrenal bez) nadir görülen primer neoplazileri; gelişen görüntüleme teknikleri ve otopsiler sayesinde günümüzde daha yüksek oranlarda tespit edilmektedir. Büyük kısmı rastlantısal olarak saptanan (insidentaloma) ve metabolik olarak inaktif (nonfonksiyonel) karakterdeki bu kitleler; lipom, myelolipom, anjiomyolipom, teratom, hemanjiom, leiomyom, schwannom, ganglionörom, primitif nöroektodermal tümör (PNET), inflamatuar myofibroblastik tümör (İMT), lenfoma ve kistler olarak sınıflandırılmaktadır. Söz konusu tümörler genellikle benign yapıda olsalar da PNET ve primer adrenal lenfoma gibi türleri yüksek malignite potansiyeline ve kötü prognoza sahiptir. Tanı süreçlerinde bilgisayarlı tomografi (CT), manyetik rezonans (MR) ve PET gibi ileri radyolojik tetkiklerin yanı sıra kesin teşhis için CD34, CD31, SMA ve S-100 gibi belirteçleri içeren histopatolojik ve immünhistokimyasal incelemeler belirleyici rol oynamaktadır. Çoğunlukla asemptomatik seyreden bu lezyonlar, büyük boyutlara ulaştıklarında çevre dokulara bası, spontan kanama, rüptür veya enfeksiyon gibi ciddi komplikasyonlara yol açabilmektedir. Tedavi yaklaşımı olarak, küçük çaplı ve belirti göstermeyen vakalar klinik takibe alınırken; bası bulgusu oluşturan, hızlı büyüyen, komplikasyon riski taşıyan veya malignite şüphesi bulunan olgularda cerrahi rezeksiyon (açık veya laparoskopik adrenalektomi) temel ve etkin tedavi yöntemi olarak uygulanmakta, malign vakalarda sürece kemoradyoterapi eklenmektedir.
The primary adrenal gland neoplasms, which are rare, are detected at higher rates today owing to advanced imaging techniques and autopsies. Mostly discovered incidentally (incidentalomas) and being metabolically inactive (nonfunctional), these masses are classified as lipoma, myelolipoma, angiomyolipoma, teratoma, hemangioma, leiomyoma, schwannoma, ganglioneuroma, primitive neuroectodermal tumor (PNET), inflammatory myofibroblastic tumor (IMT), lymphoma, and cysts. Although these tumors generally exhibit a benign structure, types such as PNET and primary adrenal lymphoma possess high malignancy potential and poor prognosis. In diagnostic processes, advanced radiological examinations like computed tomography (CT), magnetic resonance (MR), and PET play a decisive role, along with histopathological and immunohistochemical evaluations containing markers such as CD34, CD31, SMA, and S-100 for definitive diagnosis. While mostly running an asymptomatic course, these lesions can lead to serious complications including compression on surrounding tissues, spontaneous hemorrhage, rupture, or infection upon reaching large sizes. As a treatment approach, small-scale and asymptomatic cases are taken under clinical follow-up; however, in cases presenting compression symptoms, rapid growth, complication risk, or suspicion of malignancy, surgical resection (open or laparoscopic adrenalectomy) is applied as the primary and effective treatment method, with chemoradiotherapy added to the process in malignant cases.
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