Radiologically Probable Late-Onset Von Hippel-Lindau Syndrome: A Case Report

Von Hippel-Lindau (VHL) syndrome is a rare hereditary tumor syndrome characterized by multisystem involvement and variable clinical presentation. Radiological evaluation plays a pivotal role in early diagnosis and surveillance, particularly in settings where histopathological and genetic confirmation may not be feasible. We report a case of a 69-year-old male presenting with nonspecific abdominal complaints. Triple-phase contrast-enhanced CT of the thorax and abdomen demonstrated diffuse pancreatic cystosis, multiple hypervascular pancreatic lesions favoring pancreatic neuroendocrine tumors, serous cystadenomas, a hypervascular left adrenal lesion suspicious for pheochromocytoma, and multifocal left renal cortical masses suspicious for renal cell carcinoma. Based on the characteristic constellation of multiorgan thoracoabdominal imaging findings, the case was interpreted as radiologically probable late-onset VHL syndrome. Histopathological, biochemical, ophthalmologic, central nervous system (CNS) imaging, and genetic confirmation could not be obtained because the patient was lost to follow-up.