Adrenal Cortical Neoplasm with Uncertain Malignant Potential Arising in the Heterotopic Adrenal Cortex in the Liver of a Patient with Beckwith-Wiedemann Syndrome
Journal of Pathology and Translational Medicine
; : 129-135, 2019.
Article
在 En
| WPRIM
| ID: wpr-766008
Responsible library:
WPRO
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 9 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
全文:
1
索引:
WPRIM
主要主题:
Beckwith-Wiedemann Syndrome
/
Chromosomes, Human, Pair 11
/
Immunohistochemistry
/
Adrenal Cortex
/
Adrenal Gland Neoplasms
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Hepatoblastoma
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Uniparental Disomy
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Liver
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Macroglossia
研究类型:
Prognostic_studies
限制:
Humans
语言:
En
期刊:
Journal of Pathology and Translational Medicine
年:
2019
类型:
Article