J Pathol Transl Med.  2019 Mar;53(2):129-135. 10.4132/jptm.2018.11.13.

Adrenal Cortical Neoplasm with Uncertain Malignant Potential Arising in the Heterotopic Adrenal Cortex in the Liver of a Patient with Beckwith-Wiedemann Syndrome

• Kim EN
• Song DE
• Yoon HM
• Lee BH
• Kim CJ

• KMID: 2465453
• DOI: http://doi.org/10.4132/jptm.2018.11.13

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.

Keyword

Beckwith-Wiedemann syndrome; Liver; Heterotopic; Adrenal gland neoplasms

MeSH Terms

Adrenal Cortex*
Adrenal Gland Neoplasms
Beckwith-Wiedemann Syndrome*
Chromosomes, Human, Pair 11
Hepatoblastoma
Humans
Immunohistochemistry
Liver*
Macroglossia
Uniparental Disomy

Figure

• 

  Fig. 1. (A) Axial ultrasound image showing an oval-shaped hypoechoic mass in the inferior tip of the right hemi liver (arrows). Asterisk indicates the right kidney. (B) Coronal computed tomography scan showing an exophytic mass arising from the inferior tip of the right hemi liver (arrow). (C) The cut surface of the tumor is grayish yellow with focal brown pigmentation and glistening. Necrosis and hemorrhage are absent. (D) The tumor shows exophytic growth (scan view). (E) The tumor is encased in a Glisson capsule and had remaining hepatocytes (asterisk). Bile ductules (arrow) were entrapped. (F) Tumor cells with nested growth pattern had plump clear cytoplasm, distinct and smooth cell membrane, bland and small nucleus, and micro nucleoli that resemble zona fasciculata of a normal adrenal gland. (G) Hepar-1 immunohistochemical staining showing the remaining normal hepatocytes surrounding tumor cells. (H) Cytokeratin 7 immunohistochemical staining showing the entrapped bile ductules in the periphery of tumor cells. (I) The bile ducts (arrow) are not in contact with the tumor. (J) Remaining bile ducts (arrow) and hepatic parenchyma (asterisk) are pushed by the tumor. The Glisson capsule remains intact (Masson trichrome staining).

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  Fig. 2. The tumor cells show strong, diffuse immunoreactivity for CD56 (A), Vimentin (B), moderately intense immunoreactivity for Melan A (C), and weak focal immunoreactivity for inhibin α (D). (E) Synaptophysin immunoreactivity is very focal and weak. (F) Ki-67 labeling index is 13.2% in hot spot by manual count. (G, H) The tumor shows multifocal disruption of reticulin network. Electron microscopy shows numerous mitochondria with tubular cristae (I, ×2,500; J, ×10,000, black arrow) and neurosecretory granules (J, yellow arrow), which are compatible with adrenal cortical neoplasm.

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