• Advances in pediatric surgery
• /
• v.6 no.2
• /
• pp.160-165
• /
• 2000

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.

• Advances in pediatric surgery
• /
• v.5 no.2
• /
• pp.152-158
• /
• 1999

Adrenal cortical tumors are rare in adults and children. Most are malignant and functional. The principal clinical features are virilization, Cushing's syndrome, hyperaldosteronism and feminization. Recently, we treated a case of virilizing adrenal cortical tumor in a 26 month-old boy. The diagnosis was made by hormone assay, abdominal CT and tissue pathology. Right adrenalectomy was successful performed. Pathologic examination revealed an adrenal cortical adenoma with vascular invasion.

• Advances in pediatric surgery
• /
• v.1 no.1
• /
• pp.85-94
• /
• 1995

Precocious puberty is difficult to define because of the marked variation in the age at which puberty begins normally, onset of puberty before 8 years of age in girls and 9 years in boys may be considered precocious. The etiology of precocious puberty in boys is usually idiopathic, but can result from adrenal and testicular tumors. The hepatoblastoma that produces hCG is a very rare functioning tumor known to cause precocious puberty in boys. Recently, author experienced one case of virilizing adrenal cortical adenoma in 22 month-old boy, one case of adrenal cortical carcinoma in 28 month-old boy, and one case of virilizing hCG-producing hepatoblastoma in 7 year-old boy and reviewed literatures.