Ovarian thecal metaplasia of the adrenal gland in association with Beckwith-Wiedemann syndrome  

作　　者：Eslam Y Wassal Mouhammed Amir Habra Rafael Vicens Priya Rao Khaled M Elsayes 

机构地区：[1]Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center [2]Department of Endocrine Neoplasia and Hormonal Disorders,University of Texas MD Anderson Cancer Center [3]Department of Pathology, University of Texas MD Anderson Cancer Center

出　　处：《World Journal of Radiology》2014年第12期919-923,共5页世界放射学杂志（英文版）（电子版）

摘　　要：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 tumorlike mesenchymal lesion in BWS patients that lacks detailed radiological description. We report a 17-yearold 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.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 tumorlike mesenchymal lesion in BWS patients that lacks detailed radiological description. We report a 17-yearold 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.

关 键 词：OVARIAN thecal METAPLASIA ADRENAL GLAND WILMS tumor BECKWITH-WIEDEMANN syndrome Computed tomography Magnetic resonance imaging Adrenocortical carcinoma 

分 类 号：R736.6[医药卫生—肿瘤]