Von Hippel-Lindau is a genetic syndrome, comprising several variant mutations on chromosome 3, that predisposes patients to the development of benign and malignant tumors. Tough relatively common, Von Hippel-Lindau syndrome (VHL) with associated hepato-biliary and gastric outlet obstruction, and portal hypertension consequent to the mass effect of a pancreatic serous cystadenoma is a rare scenario. This manuscript reports a 41-year-old female with the aforementioned presentation who successfully underwent a palliative cholecysto- and a gastro-jejunostomy. To the knowledge of the authors, this is the only report, describing a palliative biliary decompression for VHL-related pancreatic serous cystadenoma and portal hypertension.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3999635 | PMC |
| http://dx.doi.org/10.3978/j.issn.2078-6891.2014.017 | DOI Listing |
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