[Proteus syndrome: Case report of bladder vascular malformation causing massive hematuria].

Prog Urol

Service de chirurgie viscérale pédiatrique, hôpital des Enfants de Toulouse, CHU de Toulouse, 330, avenue de Grande-Bretagne, 31059 Toulouse cedex 9, France.

Published: February 2012

Proteus syndrome is a rare, sporadic disorder consisting of disproportionate overgrowth of multiple tissues, vascular malformations, and connective tissue or epidermal nevi. Due to mosaic pattern of distribution, the phenotypes are variable and diverse. Vascular malformations are part of the major criteria used to define and diagnose this syndrome. It can involve the gastrointestinal tract, spleen, or the urinary tract but bladder malformations are rare. We report here a case of bladder vascular malformation in a 12-year-old boy known to have Proteus syndrome and review the literature on bladder malformations or tumors in this syndrome.

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http://dx.doi.org/10.1016/j.purol.2011.07.011DOI Listing

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