BACKGROUND: Proteus syndrome is a rare hamartomatous disorder characterized by the overgrowth of multiple tissues in a mosaic pattern. Tumors of genitourinary tract in Proteus syndrome are uncommon. CASE: We here report a 5-year-old girl with Proteus syndrome who developed a cystic mass in the pelvic cavity. The cyst was discovered by ultrasonographic examination and finally proved to be a unilateral ovarian dermoid cyst accompanied by an ipsilateral paratubal cyst by laparoscopic surgery. SUMMARY AND CONCLUSION: Proteus syndrome accompanied by an ovarian cyst is rare in girls. The possibilities of underlying ovarian cyst required for surgical intervention should be considered in Proteus syndrome. Copyright 2010 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.
BACKGROUND:Proteus syndrome is a rare hamartomatous disorder characterized by the overgrowth of multiple tissues in a mosaic pattern. Tumors of genitourinary tract in Proteus syndrome are uncommon. CASE: We here report a 5-year-old girl with Proteus syndrome who developed a cystic mass in the pelvic cavity. The cyst was discovered by ultrasonographic examination and finally proved to be a unilateral ovarian dermoid cyst accompanied by an ipsilateral paratubal cyst by laparoscopic surgery. SUMMARY AND CONCLUSION:Proteus syndrome accompanied by an ovarian cyst is rare in girls. The possibilities of underlying ovarian cyst required for surgical intervention should be considered in Proteus syndrome. Copyright 2010 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.