Functional Adrenocortical Adenoma in a Child with Beckwith–Wiedemann Syndrome

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 p...

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Bibliographic Details
Published in:Case reports in pediatrics 2021-08, Vol.2021, p.1-5
Main Authors: Doya, Leen Jamel, Hassan, Naya Talal, Mansour, Hanin Ahmed, Alkhalil, Mohammad Ahmad Almahmod, Alkhalil, Abdul Alrahman Almahmod, Mansour, Nada, Abdallah, Alaa
Format: Article
Language:English
Subjects:
Abdomen
Acne
Adrenal glands
Age
Androgens
Birth weight
Blood
Case Report
Case reports
Children & youth
Congenital diseases
Endocrine system
Hair
Hernias
Histopathology
Hormones
Laboratories
Literature reviews
Medical diagnosis
Medical screening
Neuroendocrine tumors
Patients
Pediatrics
Testosterone
Toddlers
Tomography
Tumors
Ultrasonic imaging
Vagina
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Summary: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.
ISSN:2090-6803
2090-6811
DOI:10.1155/2021/5570267