J Korean Assoc Pediatr Surg.  2000 Dec;6(2):160-165. 10.13029/jkaps.2000.6.2.160.

Beckwith-Wiedemann Sydrome with Left Adrenal Cortical Neoplasm

Affiliations
  • 1Department of Surgery and Pathology, St. Mary's Hospital, The Catholic University of Korea, Seoul, Korea.

Abstract

Beckwith-Wiedemann syndrome presents with multisystemic patterns of congenital anomalies and macrosomia. This syndrome was independently described 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 managed a case of Beckwith-Wiedemann syndrome with left adrenal cortical neoplasm of undetermined malignancy.

Keyword

Beckwith-Wiedemann sydrome; Adrenal cortical neoplasm

MeSH Terms

Beckwith-Wiedemann Syndrome
Hernia, Umbilical
Hypoglycemia
Macroglossia
Wilms Tumor
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