ISSN: 1300-0292 İndekslendiği Dizinler: SCIENCE CITATION INDEX EXPANDED CINAHL, Index Copernicus, Chemical Abstracts (CA), Excerpta Medica / EMBASE Dil: Türkçe, İngilizce İçerik: Orijinal Araştırma, Derleme, Editöre Mektup, Olgu Sunumu, Tıp Eğitimi, Tıbbi Kitap İncelemeleri
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A Complicated Von Hippel-lindau Case: A Case Report
Dr. Ahmet Mesrur HALEFOĞLU,a Dr. Zehra Berna ARIK,a Dr. Alper ÖZEL,a Dr. Evren YILDIZ,a Dr. Can K. ÇALIŞKANa
aRadyoloji Kliniği, Şişli Etfal Hastanesi, İSTANBUL von Hippel-Lindau (VHL) disease is an autosomal dominant disorder which causes retinal and central nervous system hemangioblastomas, endolymphatic sac tumors, renal cell carcinomas, pancreatic cysts and tumors, pheochromocytomas, and epididymal cystadenomas, along with other rare manifestations. The prevalence of VHL has been estimated to be between 1:35,000 and 1:40,000.
In this case report, we present a 48 year-old woman who manifested characteristics of this disease. She had retinal and cerebellar hemangioblastomas, left renal cell carcinoma and multiple pancreatic cysts and had undergone brain surgery and left nephrectomy. Two years later, she developed new cerebellar hemangioblastomas and right renal cell carcinoma. She has been followed up since 1998 by computed tomography and magnetic resonance imaging. Imaging modalities play a major role in the diagnosis, management and treatment of this disease.Keywords: von Hippel-Lindau Disease,magnetic resonance imaging, tomography, spiral computedTurkiye Klinikleri J Med Sci 2005, 25:753-757
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