Presentation
History of surgical resection of a small right renal cell carcinoma.
Patient Data
Multiple variable-sized pancreatic and bilateral renal cysts are noted. A small subcapsular hepatic focal lesion is also seen at segment VII possibly small cyst.
A well-defined intra-axial lesion is seen centered on the posterior aspect of the medulla oblongata appears of low signal intensity on T1, bright signal intensity on T2 with posterior enhancing intra-mural small linear area.
Case Discussion
Von Hipple-Lindau disease is a rare type of phakomatosis with various CNS, renal, pancreatic and head and neck abnormalities like hemangioblastoma, renal cysts, renal cell carcinoma, pheochromocytoma and pancreatic cysts as well as cystadenoma and cystadenocarcinoma.
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