Gastrointestinal ImagingVon hippel - lindau disease
(Eugen von Hippel, 1867 - 1939, German ophthalmologist and Arvid Lindau, 1892 - 1958, Swedish pathologist), autosomal dominant hereditary disorder, linked to chromosome 3p, and characterized by angiomata of the retina and haemangioblastoma. Other features include hypertension due to phaeochromocytoma, hypernephroma-like tumours, polycythaemia due to the renal tumour or the haemangioblastoma, haemangiomas of the spinal cord, lungs and adrenals, cysts, microcystic adenomas and neuroendocrine tumours of the pancreas, kidneys and liver, and epdidymal cystadenoma.
Pancreatic lesions may be the only abdominal localization of von Hippel Lindau disease. The extent of the cystic lesions of the pancreas varies from a few cysts to polycystic transformation of the enlarged organ (Fig.1). Microcystic adenomas (serous cystadenomas) are more rare. The cysts always contain serous fluid. No mucinous cysts have been reported. In general, cystic pancreatic lesions in von Hippel Lindau disease are asymptomatic or associated with mild symptoms only.
Imaging characteristics of both neoplasms are similar to those of other true cysts of the pancreas. Neuroendocrine tumours in von Hippel Lindau disease consist of a combination of MEN I and MEN II syndromes (see MEN syndrome). Also, see von Hippel Lindau disease adrenal involvement and von Hippel Lindau disease.
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Multiple pancreatic cysts in von Hippel - Lindau disease.
a. CT of the upper abdomen following intravenous contrast medium administration. There are numerous fluid containing cystic lesions distributed throughout the pancreas.
b. MRI, T2-weighted sequence. The cysts show a high signal intensity and are more conspicious than on CT.
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Von hippel - lindau disease, Fig.1 (a) | | Von hippel - lindau disease, Fig.1 (b) | |