Tüberoskleroz, tüm organları tutabilen ancak en sık deri, beyin, kalp, göz ve böbreği tutan benign hamartomatöz lezyonlar ile karakterize otozomal dominant bir hastalıktır. Tüberosklerozluların %40-80'inde bilateral, multipl, küçük ve sıklıkla asemptomatik renal anjiyomyolipomlar görül-mektedir. Ancak sunulan olguda olduğu gibi boyutları 20 cm'yi aşan anjiyomyolipomlar oldukça nadirdir. Boyutları büyüdükçe semptomatik olma olasılığı ve masif, hatta fatal hemorajiyle sonuçlanan spontan rüptür riski artmaktadır. Bilgisayarlı tomografi, düşük oranlardaki yağ içeriğini bile saptayabilmesi nedeniyle, renal anjiyomyolipomların tanısında ilk tercih edilen görüntüleme yöntemidir. Anahtar sözcükler: anjiyomyolipom, bilgisayarlı tomografi, tuberosklerozTuberous sclerosis is an autosomal dominant disorder characterized by hamartomas that may occur in any organ system, but most often occur in skin, brain, heart, eye and kidney. Between 40 and 80% of patients with tuberous sclerosis have renal angiomyolipomas which are often bilateral, multifocal, small and often asymptomatic. Very large renal angiomyolipoma, like the case reported here, are very rare. They are often symptomatic and have a propensity for spontaneous rupture, resulting in massive and at times, fatal hemorrhage. Because of its sensitivity in detecting small amounts of fat, computed tomography is imaging modality of choice in the diagnosis of renal angiomyolipomas.
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