Acta Médica del Centro (Jan 2008)
Masive enterorrhagia in patients suffering from hippel-lindau disease
Abstract
The Von Hippel Lindau disease is an autosomic-dominant neoplasia syndrome produced by a delection or mutation of tumor suppressor gene of chromosome 3p25. It was descripted by the German pathologist Arvid Lindau and the ophthalmologist Eugene Von Hippel, and it is considered also one of 7 000 hereditary disorders known up to the present. Our patient was submitted to a genetic study in 1990 to determine, who relatives had suffered this syndrome (by making a genealogical tree). As the antecedent was taking an operation of renal carcinoma made a year before, and this time, the patient is admitted cause presented a massive enterorrhagia, which motivated a research about him and a surgical intervention as well. The histological study demonstrated the existence of a methastasis of Clear-Cell renal carcinoma, which is a characteristic lesion, when Occurs this Syndrome.