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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.
Artículo en Inglés | WPRIM | ID: wpr-766008
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.
Asunto(s)

Texto completo: Disponible Índice: WPRIM (Pacífico Occidental) Asunto principal: Síndrome de Beckwith-Wiedemann / Cromosomas Humanos Par 11 / Inmunohistoquímica / Corteza Suprarrenal / Neoplasias de las Glándulas Suprarrenales / Hepatoblastoma / Disomía Uniparental / Hígado / Macroglosia Tipo de estudio: Estudio pronóstico Límite: Humanos Idioma: Inglés Revista: Journal of Pathology and Translational Medicine Año: 2019 Tipo del documento: Artículo

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Texto completo: Disponible Índice: WPRIM (Pacífico Occidental) Asunto principal: Síndrome de Beckwith-Wiedemann / Cromosomas Humanos Par 11 / Inmunohistoquímica / Corteza Suprarrenal / Neoplasias de las Glándulas Suprarrenales / Hepatoblastoma / Disomía Uniparental / Hígado / Macroglosia Tipo de estudio: Estudio pronóstico Límite: Humanos Idioma: Inglés Revista: Journal of Pathology and Translational Medicine Año: 2019 Tipo del documento: Artículo