Periampullary and Pancreatic Metastatic Disease of Renal Cell Carcinoma: An Underdiagnosed Disease!

Abstract

Purpose: The National Cancer Institute reports high incidence of renal cell carcinoma (RCC) in the United States compared to other regions. However, pancreatic and periampullary metastasis are uncommon when only 17% of the RCC cases metastasize overall. We herein present a case series of four patients with periampullary or pancreatic metastatic disease following complete resection of RCC, evaluating their occurrences and outcomes. We reviewed the cases of four male patients retrospectively, mean age 75 years (range 65 to 87 years) who had a previous history of nephrectomy for RCC. They experienced recurrence with periampullary (two patients) or pancreatic (two patients) metastatic disease between 0 to 108 months (mean time 41.5 months) following primary tumor resection. In patients with periampullary metastasis, one had asymptomatic presentation with progressive dilatation of the pancreatic duct noted on surveillance CT scans. The other patient had iron deficiency anemia and melena with EGD findings of large fungating infiltrative ulcerative mass in the area of duodenal papilla (the only patient with metastasis to other sites: lungs and colon). As for those with pancreatic metastasis, one patient presented with hematuria and abdominal pain and was found to have pancreatic metastasis at the time of RCC diagnosis. The other patient was admitted for further workup of a mass in the pancreatic tail upon surveillance. Pathologic findings included high-grade renal cell carcinoma in the metastatic foci. Management of such patients included distal pancreatectomy in two patients without chemoradiation, one is awaiting Whipple procedure and received four cycles of sunitinib, while the last is a poor surgical candidate and received aminocaproic acid. Three patients are still alive to date. Optimal management is challenging given the very high risk of delayed relapse following tumor resection of the localized disease, leaving such cases with a very poor prognosis. Therefore to enhance survival, it is imperative to have careful stage-dependent surveillance in patients who have undergone a prior resection of RCC. We emphasize the importance of raising awareness for this unusual presentation. Disease recurrence as a pancreatic mass or hepatobiliary ductal dilatation might be more frequent than previously reported.