BILATERAL BENIGN HAEMORRHAGIC ADRENAL CYSTS IN BECKWITH-WIEDEMANN SYNDROME: A CASE REPORT

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Main Author: Fatima Mbarki , Khadija Skalli , Driss Oukachou and Fouzia Hmami
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Published: Zenodo 2025
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author Fatima Mbarki , Khadija Skalli , Driss Oukachou and Fouzia Hmami
author_facet Fatima Mbarki , Khadija Skalli , Driss Oukachou and Fouzia Hmami
contents <p>Beckwith-Wiedemann syndrome (BWS) is a genetic proliferative disorder, often associated with hyperplasia of various organs, including the adrenal glands.(1) Benign hemorrhagic adrenal cysts are a relatively common form of adrenal mass observed in patients with this syndrome, although they are typically asymptomatic. However, their presence requires careful monitoring due to the potential risks of complications and progression.(2) We report a case of bilateral adrenal cystic masses detected during a routine third-trimester prenatal ultrasound, associated with macrosomia and macroglossia at birth. The diagnosis of Beckwith-Wiedemann syndrome was suspected in our patient. Prenatal and postnatal ultrasounds, magnetic resonance imaging (MRI), and biological assessments failed to establish the origin of these adrenal masses. Differential diagnoses considered included bilateral cystic neuroblastoma, bilateral cystic lymphangioma, bilateral cystic adrenal cortical adenoma, and duplication of the renal-ureteral system. A laparotomy was performed two months after birth due to the large size of the tumor and suspicion of its tumorigenic origin. Histopathological examination revealed a cystic adrenal hematoma, with no signs of malignancy. The diagnosis of bilateral benign hemorrhagic macrocystic adrenal component associated with Beckwith-Wiedemann syndrome was confirmed. A multidisciplinary approach, including close radiological and biological monitoring, was implemented.</p> <p> </p>
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spellingShingle BILATERAL BENIGN HAEMORRHAGIC ADRENAL CYSTS IN BECKWITH-WIEDEMANN SYNDROME: A CASE REPORT
Fatima Mbarki , Khadija Skalli , Driss Oukachou and Fouzia Hmami
Beckwith-Wiedemann Syndrome-Bilateral Haemorragique Adrenal cysts
<p>Beckwith-Wiedemann syndrome (BWS) is a genetic proliferative disorder, often associated with hyperplasia of various organs, including the adrenal glands.(1) Benign hemorrhagic adrenal cysts are a relatively common form of adrenal mass observed in patients with this syndrome, although they are typically asymptomatic. However, their presence requires careful monitoring due to the potential risks of complications and progression.(2) We report a case of bilateral adrenal cystic masses detected during a routine third-trimester prenatal ultrasound, associated with macrosomia and macroglossia at birth. The diagnosis of Beckwith-Wiedemann syndrome was suspected in our patient. Prenatal and postnatal ultrasounds, magnetic resonance imaging (MRI), and biological assessments failed to establish the origin of these adrenal masses. Differential diagnoses considered included bilateral cystic neuroblastoma, bilateral cystic lymphangioma, bilateral cystic adrenal cortical adenoma, and duplication of the renal-ureteral system. A laparotomy was performed two months after birth due to the large size of the tumor and suspicion of its tumorigenic origin. Histopathological examination revealed a cystic adrenal hematoma, with no signs of malignancy. The diagnosis of bilateral benign hemorrhagic macrocystic adrenal component associated with Beckwith-Wiedemann syndrome was confirmed. A multidisciplinary approach, including close radiological and biological monitoring, was implemented.</p> <p> </p>
title BILATERAL BENIGN HAEMORRHAGIC ADRENAL CYSTS IN BECKWITH-WIEDEMANN SYNDROME: A CASE REPORT
topic Beckwith-Wiedemann Syndrome-Bilateral Haemorragique Adrenal cysts
url https://doi.org/10.21474/IJAR01/20378