Von Hippel-Lindau syndrome

Case contributed by Nguyen Duy Hung
Diagnosis certain


History of tumor resection at the posterior fossa

Patient Data

Age: 25 years
Gender: Female

Multiple enhancing intra-axial lesions in the posterior fossa. Cystic lesion with no mural nodule also detected in the right cerebellum.


Cystic mass with a mural nodule was detected in the conus medullaris. The mural nodule showed vivid enhancement on post-contrast images. Some small multiple enhancing lesions which are similar to those in the brain were also identified in this area.


A homogenous,well-circumscribe mass at the pancreatic head was observed. The mass vividly enhanced on post-contrast images. No fat infiltration or regional lymphadenopathy was found.

Case Discussion

The pathological result of the posterior fossa tumor was hemangioblastoma.

The pancreatic mass was surgically removed and pathologically proven endocrine tumor.

Regarding the pathological result of the pancreatic and posterior fossa masses, the imaging features on MRI of the brain and spine are characteristic of Von Hippel-Lindau syndrome.

Besides CSN and pancreatic manifestations, Von Hippel-Lindau syndrome is characterized by tumors in other organs such as renal cell carcinomas, retinal hemangioblastomas, or liver cysts.

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