Background: Von Hippel-Lindau (VHL) disease may occur at various localisations which can be both intra- and extrapancreatic as well as challenging to diagnose by medical imaging.
Case report: A positron emission tomography/magnetic resonance imaging in a 40-year-old woman was performed to monitor a haemangioblastoma. Additionally, it showed findings which were considered to be a pancreatic neuroendocrine tumour (pNET) and retroumbilical metastasis. The suspected metastasis was laparoscopically resected; however, pathological evaluation did not lead to a clear categorisation. Consequently, the pancreatic head was resected in which a pNET and various acinar cell cystadenomas were found.
Conclusion: Diagnostic and therapy of advanced VHL disease can be difficult; if in doubt, a surgical approach may establish clarity.
Keywords: Acinar cell cystadenoma; Pancreatic neuroendocrine tumour, pNET; von Hippel-Lindau disease.