Cemil YAĞCI, Suat FİTÖZ, Serdar AKYAR, E. Nisa ÜNLÜ, Çetin ATASOY

Tüberoklerozda dev renal anjiyomyolipomlar: Bilgisayarlı tomografi bulguları

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 görülmektedir. 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.

Giant renal angiomyolipomas in tuberous sclerosis: Computed tomography findings

Tuberous 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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1. Ashebu SD, Dahniya MH, Elshebiny YH et al. Giant bleeding renal angiomyolipoma: diagnosis and management. Australas Radiol 2002; 46:115-118.
2. Katz DS, Poster RB. Massif renal angiomyolipoma in tuberous sclerosis. Clin Imaging 1997; 21:200-202.
3- Caldemeyer KS, Mirowski GW. Tuberous sclerosis. Part I. Clinical and central nervous system findings. J Am Acad Dermatol 2001; 45:448-449.
4. Evans JC, Curtis C. The radiological appearances of tuberous sclerosis. Br J Radiol 2000; 73:91-98.
5. Pui MH, Kong HL, Choo HE Bone changes in tuberous sclerosis mimicking metastases. Australas Radiol 1996; 40:77-79.
6. Bernauer TA, Mirowski GW, Caldemeyer KS. Tuberous sclerosis. Part II. Musculoskeletal and visceral findings. J Am Acad Dermatol 2001; 45:450-452.