Aralik 2010
Pancreatic neuroendocrine tumor and renal cell carcinoma in a patient: Von Hippel-Lindau Disease
Pankreatik nöroendokrin tümör ve böbregin seffaf hücreli karsinomu: Von Hippel-Lindau Hastaligi
- Ana Sayfa
- Sayılar
- Aralik 2010
- Pancreatic neuroendocrine tumor and renal cell carcinoma in a patient: Von Hippel-Lindau Disease...
Özet
Von Hippel Lindau hastaligi 3. kromozomdaki Von Hippel Lindau tümör supresör genindeki bir mutasyona bagli olarak otozomal dominant (kalitim ?geçis gösteren) multisistemik bir kanser sendromudur. Von Hippel Lindau hastaliginda major lezyonlar santral sinir sisteminde ve retinadaki hemanjioblastomlar, böbregin seffaf hücreli (clear cell) karsinomu, feokromasitoma, pankreas tümörleri, epididimal kist adenomlar, endolenfatik kese tümörleri, karsinoid tümörler ve böbregin, pankreasin, epididimisin kist adenomlaridir. Von Hippel Lindau hastalarinin bir çogunda basit kistler, mikrokistik adenomlar, pankreatik nöroedokrin tümörler ve böbrek karsinomunun pankreas metastazlari olmak üzere degisik pankreatik lezyonlar görülebilmektedir. Pankreatik nöroedokrin tümörlerin hipervasküler tümörler ve özellikle de böbregin seffaf hücreli karsinomu ve mikrokistik adenomlar gibi seffaf hücreli tümörlerden ayirici tanisi yapilmalidir. Burada pankreas nöroendokrin tümörü ile böbregin seffaf hücreli karsinomu olan bir Von Hippel Lindau hastaligi olgusu sunulmaktadir.
Abstract
Von Hippel-Lindau disease is an autosomal dominant multisystemic cancer syndrome due to a mutation of the Von Hippel-Lindau disease tumor suppressor gene on chromosome 3. The major lesions in Von Hippel-Lindau disease disease include hemangioblastomas in the central nervous system and retina, clear cell renal cell carcinomas, pheochromocytomas, pancreatic tumors, epididymal cystadenomas, endolymphatic sac tumors, carcinoid tumors, and multiple cysts of the kidney, pancreas and epididymis. Most patients have pancreatic involvement in Von Hippel-Lindau disease disease. Pancreatic disease of Von Hippel-Lindau disease includes benign cysts, microcystic adenomas, pancreatic neuroendocrine tumors , and pancreatic metastases of renal cell carcinoma. Pancreatic neuroendocrine tumors should be differentiated from other hypervascular tumors and especially with clear cell morphology, such as renal cell carcinoma and microcystic adenomas. Herein, we report a patient with the diagnosis of Von Hippel-Lindau disease who had malignant neuroendocrine tumorof the pancreas and renal adenocarcinoma.
