Published online Dec 28, 2014. doi: 10.4329/wjr.v6.i12.919
Revised: November 2, 2014
Accepted: November 17, 2014
Published online: December 28, 2014
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.
Core tip: This paper represents radiological features of the unique adrenal pathology in a premenopausal woman with Beckwith Wiedemann syndrome (BWS), although adrenal masses in BWS should raise the suspicion for adrenocortical carcinoma, yet other adrenal tumors including ovarian thecal metaplasia should be considered.