2020 Volume 53 Issue 1 Pages 69-76
A 39-year-old woman was referred to our department because of a pancreatic head tumor, diffuse cystic lesions in the pancreas, and a right renal tumor detected in a medical check-up. The pancreatic head tumor was diagnosed as pancreatic neuroendocrine tumor (PanNET) G1 by endoscopic ultrasound-guided fine-needle aspiration, and the renal tumor was clinically diagnosed as renal cell carcinoma (RCC). The patient was suspected of suffering from von Hippel-Lindau (VHL) disease. Genetic testing found mutation in exon 2 of VHL gene which resulted in definitive diagnosis of VHL disease. Pancreaticoduodenectomy and right partial nephrectomy were performed in one stage. Histopathological examinations revealed PanNET G1 with regional lymph node metastasis, serous cystadenoma (SCA), and clear cell renal cell carcinoma. Here, we describe a rare case of a VHL patient with PanNET, SCA and RCC.
