Proteus syndrome: report of a case with recurrent abdominal lipomatosis.
Proteus syndrome (PS) is an extremely rare congenital hamartomatous syndrome that was first delineated by Cohen and Hayden (1). The estimated prevalence is less than 1 per 1,000,000 live births (2). It is a sporadic disorder that causes overgrowth of multiple tissues, especially bone, fat, and other connective tissues in a patchy or mosaic pattern. Subcutaneous as well as internal lipomas that may grow to an enormous size are frequently observed. Nevertheless, among the internal lipomas, abdominal lipomatosis is rare (3), with less than 15 cases reported. Herein, we report the first patient described with this distinctive syndrome associated with lipomatosis involving the epiploon.
Furquim
Isabel
I
Unidade de Genética-Instituto da Criança-HCFMUSP. belmosca@hotmail.com
Honjo
Rachel
R
Bae
Ricardo
R
Andrade
Wagner
W
Santos
Maria
M
Tannuri
Uenis
U
Kim
Chong
C
D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007813:Laparotomy / Q000379:methods; D008068:Lipomatosis / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D009852:Omentum / Q000473:pathology; D010532:Peritoneal Diseases / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D016715:Proteus Syndrome / Q000150:complications / Q000175:diagnosis*; D035583:Rare Diseases; D012008:Recurrence; D012086:Reoperation; D018570:Risk Assessment; D016896:Treatment Outcome