Open AccessOvarian thecal metaplasia of the adrenal gland in association with Beckwith-Wiedemann syndrome
Author(s) -
Eslam Wassal,
Mouhammed Amir Habra,
Rafael A. Vicens,
Priya Rao,
Khaled M. Elsayes
Publication year - 2014
Publication title -
world journal of radiology
Language(s) - English
Resource type - Journals
ISSN - 1949-8470
DOI - 10.4329/wjr.v6.i12.919
Subject(s) - medicine , beckwith–wiedemann syndrome , pathology , adrenal gland , adrenocortical carcinoma , metaplasia , biochemistry , gene expression , chemistry , dna methylation , gene
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.