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Case Reports
. 2021 Aug 6:2021:5570267.
doi: 10.1155/2021/5570267. eCollection 2021.

Functional Adrenocortical Adenoma in a Child with Beckwith-Wiedemann Syndrome

Leen Jamel Doya  1 , Naya Talal Hassan  2 , Hanin Ahmed Mansour  1 , Mohammad Ahmad Almahmod Alkhalil  3 , Abdul Alrahman Almahmod Alkhalil  4 , Nada Mansour  1 , Alaa Abdallah  3
Affiliations
Case Reports

Functional Adrenocortical Adenoma in a Child with Beckwith-Wiedemann Syndrome

Leen Jamel Doya et al. Case Rep Pediatr. .

Abstract

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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Conflict of interest statement

All of the authors declare that they have no conflicts of interest.

Figures

Figure 1
Figure 1
An abdominal ultrasound showed a well-defined rounded hypoechoic lesion with multiple tiny calcifications in the left suprarenal region measured 37 × 27 × 25 mm.
Figure 2
Figure 2
Grossly, encapsulated mass, no invasion to surroundings, size 5 × 4 × 2.8 cm.
See this image and copyright information in PMC

References

    1. Cohen M. M. Beckwith-Wiedemann syndrome: historical, clinicopathological, and etiopathogenetic perspectives. Pediatric and Developmental Pathology. 2005;8(3):287–304. doi: 10.1007/s10024-005-1154-9. - DOI - PubMed
    1. Weksberg R., Shuman C., Beckwith J. B. Beckwith-Wiedemann syndrome. European Journal of Human Genetics. 2010;18(1):8–14. doi: 10.1038/ejhg.2009.106. - DOI - PMC - PubMed
    1. Thorburn M. J., Wright E. S., Miller C. G., Smith-Read E. H. Exomphalos-macroglossia-gigantism syndrome in Jamaican infants. Archives of Pediatrics and Adolescent Medicine. 1970;119(4):316–321. doi: 10.1001/archpedi.1970.02100050318006. - DOI - PubMed
    1. Sotelo-Avila C., Gooch W. M. Neoplasms associated with Beckwith-Wiedermann syn-drome. Perspectives in Pediatric Pathology. 1976;3(2):255–272. - PubMed
    1. Brioude F., Kalish J. M., Mussa A., et al. Clinical and molecular diagnosis, screening and management of Beckwith-Wiedemann syndrome: an international consensus statement. Nature Reviews Endocrinology. 2018;14(4):229–249. doi: 10.1038/nrendo.2017.166. - DOI - PMC - PubMed

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