Abstract
Von Hippel-Lindau (VHL) disease is a benign or malignant tumor syndrome which involves multiple systems and organs. Recently, a patient was diagnosed and hospitalized with an initial clinical symptom of pancreatic multiple cystic space-occupying. Multidisciplinary examination and consultation confirmed that there were hemangioblastomas in cerebella, spinal cord and retina, but with no clinical symptoms; therefore, it was diag-nosed as VHL disease. According to previous case reports, only a few single cases revealed lesions in so many parts of the central nervous system with typical imaging manifestations. We present an overview and aim to improve the diagnosis of VHL disease with the initial clinical symptom of pancreatic lesions. Key words: VHL disease; Hemangioblastoma; Pancreatic cyst
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