title: Proteus syndrome: report of intra-abdominal lipomatosis.
abstract: Proteus syndrome (PS) is an extremely rare sporadic disorder that manifests as an asymmetric, disproportionate overgrowth of any connective tissues, such as bone, fat, or epidermal nevi, in a mosaic or patchy pattern. This hamartoneoplastic syndrome was first described by Cohen and Hayden. Its prevalence is approximately 1 per 1,000,000 live births, and intra-abdominal expansion has been reported in no more than 20 cases in the literature. The phenotypes of the patients differ because of the variation in the pattern of the overgrowths, making diagnosis difficult. Extremely large subcutaneous lipomas and internal lipomas, which occur rarely, are one of the presentation phenotypes. Here, we present the second patient in the literature with PS involving the epiploon.
authors.affiliation: Department of Pediatric Surgery, Sisli Etfal Education and Research Hospital, Istanbul-Sisli, Istanbul, Turkey.
authors.forename: Basak
authors.initials: B
authors.lastname: Erginel
authors.affiliation: Department of Pediatric Surgery, Sisli Etfal Education and Research Hospital, Istanbul-Sisli, Istanbul, Turkey.
authors.forename: Melih
authors.initials: M
authors.lastname: Akin
authors.affiliation: Department of Pediatric Surgery, Sisli Etfal Education and Research Hospital, Istanbul-Sisli, Istanbul, Turkey.
authors.forename: Abdullah
authors.initials: A
authors.lastname: Yildiz
authors.affiliation: Department of Pediatric Surgery, Sisli Etfal Education and Research Hospital, Istanbul-Sisli, Istanbul, Turkey.
authors.forename: Cetin
authors.initials: C
authors.lastname: Karadag
authors.affiliation: Department of Pediatric Surgery, Sisli Etfal Education and Research Hospital, Istanbul-Sisli, Istanbul, Turkey.
authors.forename: Nihat
authors.initials: N
authors.lastname: Sever
authors.affiliation: Department of Pathology, Sisli Etfal Education and Research Hospital, Istanbul-Sisli, Istanbul, Turkey.
authors.forename: Canan
authors.initials: C
authors.lastname: Tanik
authors.affiliation: Department of Radiology, Sisli Etfal Education and Research Hospital, Istanbul-Sisli, Istanbul, Turkey.
authors.forename: Mehmet
authors.initials: M
authors.lastname: Erturk
authors.affiliation: Department of Pediatric Surgery, Sisli Etfal Education and Research Hospital, Istanbul-Sisli, Istanbul, Turkey.
authors.forename: Ali Ihsan
authors.initials: AI
authors.lastname: Dokucu
keywords: Proteus; intra-abdominal; lipomatosis