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Adrenocortical adenoma in a Sudanese girl with Beckwith-Wiedemann syndrome

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-Wiede...

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Detalles Bibliográficos
Autores principales: Elnaw, Eman Abdalla Ali, Abdalla, Awad Rhmattalla, Abdullah, Mohamed Ahmed
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6873727/
https://www.ncbi.nlm.nih.gov/pubmed/31768183
http://dx.doi.org/10.1186/s13633-019-0068-7
Descripción
Sumario: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.