RESUMO
Synchronous pancreatic neuroendocrine tumors and renal cell cancer are extremely rare. Von-Hipple-Landau syndrome is a major association. A 43-year-old male patient with left upper quadrant pain and significant weight loss was diagnosed with a synchronous pancreatic tail neuroendocrine tumor with solitary splenic metastasis and a clear-cell renal cell carcinoma of the left kidney. Sonography and a computed tomography scan of the abdomen showed a complex exophytic left renal mass and a necrotic lesion limited to the spleen. Although not apparent on preoperative imaging, distal pancreatic mass was also discovered intraoperatively. Subsequently, left radical nephrectomy, splenectomy, and distal pancreatectomy were performed, and the synchronous primaries and splenic metastasis were confirmed histopathologically. This case is unique in that it demonstrates multiple extremely rare events occurring simultaneously, namely pancreatic and kidney primaries, as well as solitary splenic metastasis.