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doi: https://doi.org/10.4132/jptm.2018.11.13    [Epub ahead of print]
Adrenal Cortical Neoplasm with Uncertain Malignant Potential Arising in the Heterotopic Adrenal Cortex in the Liver of a Patient with Beckwith-Wiedemann Syndrome: A Case RTeport
Eun Na Kim1, Dong Eun Song1, Hee Mang Yoon2, Beom Hee Lee3,4, Chong Jai Kim1
1Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
2Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
3Medical Genetics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, Seoul, South Korea
4Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, Seoul, South Korea
Corresponding Author: Chong Jai Kim ,Tel: +82-2-3010-4516, Fax: +82-2-472-7898, Email: ckim@amc.seoul.kr
Received: September 9, 2018;  Revised: November 10, 2018  Accepted: November 12, 2018.  Published online: November 26, 2018.
ABSTRACT
Patients with Beckwith-Wiedemann syndrome (BWS) are predisposed to developing embryonal tumors, with hepatoblastoma being the most common type. Our patient showed hemihypertrophy, macroglossia, and paternal uniparental disomy in chromosome 11 and was diagnosed with BWS. When the patient was nine months old, a 2.5×1.5 cm oval hypoechoic exophytic mass was detected in the inferior tip of his right liver. Preoperative imaging identified it as hepatoblastoma; however, histologic, immunohistochemistry and electron microscopic findings were compatible with adrenal cortical neoplasm with uncertain malignant potential. The origin of the adrenal tissue seemed to be heterotopic. Here, we describe for the first time an adrenal cortical neoplasm with uncertain malignant potential arising in the heterotopic adrenal cortex located in the liver of a patient with BWS.
Key Words: Beckwith-Wiedemann syndrome, Liver, Heterotopic, Adrenal neoplasm
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