Beckwith-Wiedemann sydrome with left adrenal cortical neoplasm .
- Author:
Jae Hee JUNG
;
Jae Kyoung KO
;
Young Tack SONG
- Publication Type:Original Article
- Keywords:
Beckwith-Wiedemann sydrome;
Adrenal cortical neoplasm
- MeSH:
Beckwith-Wiedemann Syndrome;
Hernia, Umbilical;
Hypoglycemia;
Macroglossia;
Wilms Tumor
- From:Journal of the Korean Association of Pediatric Surgeons
2000;6(2):160-165
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Beckwith-Wiedemann sydrome is a multisystemic pattern of congenital anomalies with overgrowth. This syndrome is first described independently by Beckwith in 1963 and by Wiedemann in 1964. There is wide spectrum of clinical manifestations, including prenatal or postnatal overgrowth, neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, hemihypertrophy and a predisposition for embryonal tumors, most frequently Wilms' tumor. We experienced a case of Beckwith-Wiedemann syndrome who developed left adrenal cortical neoplasm of indeterminate malignant potential.
