Adrenocortical adenoma in a Sudanese girl with Beckwith-Wiedemann syndrome.

ABSTRACT: Background:We report a case of right adrenocortical adenoma in a girl with features suggestive of Beckwith Wiedemann syndrome to show the importance of tumor surveillance in patients with Beckwith Wiedemann syndrome. Case presentation:A 4-years-old female with features suggestive of Beckwith-Wiedemann syndrome presented with 9?months history of virilization. Hormonal investigations results showed high levels of testosterone (2.3?ng/ml, normal values 0.1-0.4?ng/ml), and DHEAS (73?ng/ml normal values 1-6?ng/ml) with normal cortisol level. Computed tomography revealed a right adrenal mass. She underwent right adrenalectomy. Histopathological examination of the resected adrenal gland showed adrenocortical adenoma. Her postoperative evaluation showed a normal testosterone level. Conclusion:Adrenocortical neoplasms though rare in children are well documented in Beckwith-Wiedemann syndrome patients. So tumor surveillance protocol should be employed, even in a resource-limited setting for early tumor detection and a better outcome.

SUBMITTER: Elnaw EAA 

PROVIDER: S-EPMC6873727 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature