Süleyman KARAKÖSE, Murat AKYILDIZ, Meliha ZENGİN, Funda YILMAZ, Oktay TEKEŞİN

Pankreatik nöroendokrin tümör ve böbreğin şeffaf hücreli karsinomu: Von Hippel-Lindau Hastalığı

Von Hippel Lindau hastalığı 3. kromozomdaki Von Hippel Lindau tümör supresör genindeki bir mutasyona bağlı olarak otozomal dominant (kalıtım geçiş gösteren) multisistemik bir kanser sendromudur. Von Hippel Lindau hastalığında major lezyonlar santral sinir sisteminde ve retinadaki hemanjioblastomlar, böbreğin şeffaf hücreli (clear cell) karsinomu, feokromasitoma, pankreas tümörleri, epididimal kist adenomlar, endolenfatik kese tümörleri, karsinoid tümörler ve böbreğin, pankreasın, epididimisin kist adenomlarıdir. Von Hippel Lindau hastalarının bir çoğunda basit kistler, mikrokistik adenomlar, pankreatik nöroedokrin tümörler ve böbrek karsinomunun pankreas metastazları olmak üzere değişik pankreatik lezyonlar görülebilmektedir. Pankreatik nöroedokrin tümörlerin hipervasküler tümörler ve özellikle de böbreğin şeffaf hücreli karsinomu ve mikrokistik adenomlar gibi şeffaf hücreli tümörlerden ayırıcı tanısı yapılmalıdır. Burada pankreas nöroendokrin tümörü ile böbreğin şeffaf hücreli karsinomu olan bir Von Hippel Lindau hastalığı olgusu sunulmaktadır.

Anahtar Kelimeler:

Von Hippel Lindau Hastalığı, pankreas, böbreğin şeffaf hücreli karsinomu

Pankreatik nöroendokrin tümör ve böbreğin şeffaf hücreli karsinomu: Von Hippel-Lindau Hastalığı

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.

Keywords:

Von Hippel-Lindau disease, pancreatic involvement, renal cell carcinoma,

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