Presentation
Abdominal discomfort, Irritability or confusion, Dizziness
Patient Data
Huge cystic mass with peripheral calcifications of the upper pole left kidney. No tissular portion is noted or enhanced.
There is a small lower pole left kidney mass with solid and cystic components.
Three pancreatic head nodules with vivid enhancement on arterial phase, compatible with neuroendocrine tumors.
The portal phase is not available.
Huge cystic mass of the upper pole of the left kidney, with heterogeneous content (intracystic debris) without solid portion or enhancement after contrast.
Multiple pancreatic simple cysts
Three nodular formations of the pancreatic head with vivid enhancement in the arterial phase, persistent in the portal phase without compression or dilatation of the CBD and Wirsung duct, compatible with a neuroendocrine tumor (probably insulinoma).
A solid necrotizing nodule of the lower left kidney pole, very suspicious of RCC
Left cerebellar cystic mass with mural nodule vividly enhancing after contrast administration.
Note that there is no enhancement of the cyst wall.
peritumoral edema in FLAIR images.
This is a highly suggestive appearance of a hemangioblastoma.
Case Discussion
This is a typical case of a Von Hippel-Lindau disease (possibly type 1).
Von Hippel-Lindau (vHL) disease is characterized by the development of numerous benign and malignant tumors in different organs.
Organ involvement occurs in the following:
Head and neck: Retinal hemangioblastoma- endolymphatic sac tumor
Renal: cysts, angiomyolipoma, renal cell carcinoma
Liver: cysts
Pancreas: cysts, neuroendocrine tumor, serous cystadenoma
adrenal: pheochromocytomas:
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