title: Ovarian thecal metaplasia of the adrenal gland in association with Beckwith-Wiedemann syndrome.
abstract: Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome associated with increased risk to develop malignancies including adrenocortical carcinoma. Ovarian thecal metaplasia of the adrenal gland is a rare tumor-like mesenchymal lesion in BWS patients that lacks detailed radiological description. We report a 17-year-old female patient with BWS, associated with bilateral Wilms tumor, hepatic hemangiomatosis, pancreatic neuroendocrine tumor, and a phyllodes tumor of the right breast. Surveillance abdominal ultrasound identified a right adrenal mass that was further characterized by computed tomography and magnetic resonance imaging. Radiologically, this mass displayed features that overlap with adrenocortical carcinoma and pheochromocytoma but after pathological examination this proved to be an ovarian thecal metaplasia of the adrenal gland. Adrenal masses in BWS should raise the suspicion for adrenocortical carcinoma though other adrenal tumors including ovarian thecal metaplasia can be seen in these patients.
authors.affiliation: Eslam Y Wassal, Rafael Vicens, Khaled M Elsayes, Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States.
authors.forename: Eslam Y
authors.initials: EY
authors.lastname: Wassal
authors.affiliation: Eslam Y Wassal, Rafael Vicens, Khaled M Elsayes, Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States.
authors.forename: Mouhammed Amir
authors.initials: MA
authors.lastname: Habra
authors.affiliation: Eslam Y Wassal, Rafael Vicens, Khaled M Elsayes, Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States.
authors.forename: Rafael
authors.initials: R
authors.lastname: Vicens
authors.affiliation: Eslam Y Wassal, Rafael Vicens, Khaled M Elsayes, Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States.
authors.forename: Priya
authors.initials: P
authors.lastname: Rao
authors.affiliation: Eslam Y Wassal, Rafael Vicens, Khaled M Elsayes, Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States.
authors.forename: Khaled M
authors.initials: KM
authors.lastname: Elsayes
keywords: Adrenal gland; Adrenocortical carcinoma; Beckwith-Wiedemann syndrome; Computed tomography; Magnetic resonance imaging; Ovarian thecal metaplasia; Wilms tumor