Monday, September 24, 2012

What is Von Lindau Hipple Syndrome

Von Hipple Lindau Syndrome is a syndrome associated with renal cell carcinoma. It is characterised by formation of tumors and cysts throughout the body. These tumors and cysts are usually identified during the adulthood. The incidence of this syndrome is 1 in 36 000 people.

The tumors associated with Von Hipple Lindau Syndrome can be either malignant or non-malignant. These tumors can occur in various organs in the body. This syndrome is characterised by having a type of tumor called haemangioblastoma. It is a benign tumour, but can give rise threatening complications.They can occur in the retina, brain and spinal cord and cause serious neurological deficiencies.

The syndrome can give rise to tumours in the kidneys called clear cell renal cell carcinoma. It can also produce adrenal gland tumours callled pheochromocttoma. Pheochromacytoma are benign, but they can produce life threatening coomplications such as very high blood pressure. The syndrome can also present with pancreatic neuroendocrine tumours. Endolymphatic sac tunours are also seen in people with this sndrome.

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