Open AccessFunctional Adrenocortical Adenoma in a Child with Beckwith–Wiedemann Syndrome
Author(s) -
Leen Jamel Doya,
Naya Talal Hassan,
Hanin Ahmed Mansour,
Mohammad Ahmad Almahmod Alkhalil,
Abdul Alrahman Almahmod Alkhalil,
Nada Mansour,
Alaa Abdallah
Publication year - 2021
Publication title -
case reports in pediatrics
Language(s) - English
Resource type - Journals
eISSN - 2090-6803
pISSN - 2090-6811
DOI - 10.1155/2021/5570267
Subject(s) - beckwith–wiedemann syndrome , medicine , hemihypertrophy , adrenocortical adenoma , adrenal adenoma , male pseudohermaphroditism , abdominal mass , gigantism , adenoma , endocrinology , pathology , biochemistry , gene expression , chemistry , dna methylation , gene
Beckwith–Wiedemann syndrome (BWS) is a rare congenital condition characterized by complex overgrowth of different body parts. Children with Beckwith–Wiedemann syndrome, particularly those with hemihypertrophy, experience an increased risk of developing benign and malignant tumors. This case report presents an 18-month-old girl with features suggestive of Beckwith–Wiedemann syndrome who developed pubic hair, high levels of testosterone, and DHEAS with normal cortisol and progesterone levels. Computed tomography revealed a left adrenal mass. Histopathological examination of the resected mass showed an adrenocortical tumor. Her postoperative evaluation showed normal testosterone and DHEAS levels. Early diagnosis and detection of intra-abdominal neoplasms in infants with Beckwith–Wiedemann syndrome are essential to avoid serious clinical complications.
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