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Case Study
- Adrenal Cortical Neoplasm with Uncertain Malignant Potential Arising in the Heterotopic Adrenal Cortex in the Liver of a Patient with Beckwith-Wiedemann Syndrome
- Eun Na Kim, Dong Eun Song, Hee Mang Yoon, Beom Hee Lee, Chong Jai Kim
- J Pathol Transl Med. 2019;53(2):129-135. Published online November 26, 2018
- DOI: https://doi.org/10.4132/jptm.2018.11.13
- 5,212 View
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- 2 Web of Science
- 2 Crossref
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Abstract
PDF - 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.
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Citations
Citations to this article as recorded by
- Beckwith–Wiedemann syndrome: Clinical, histopathological and molecular study of two Tunisian patients and review of literature
Hela Sassi, Yasmina Elaribi, Houweyda Jilani, Imen Rejeb, Syrine Hizem, Molka Sebai, Nadia Kasdallah, Habib Bouthour, Samia Hannachi, Jasmin Beygo, Ali Saad, Karin Buiting, Dorra H’mida Ben‐Brahim, Lamia BenJemaa
Molecular Genetics & Genomic Medicine.2021;[Epub] CrossRef - Adrenocortical Tumors in Children With Constitutive Chromosome 11p15 Paternal Uniparental Disomy: Implications for Diagnosis and Treatment
Emilia Modolo Pinto, Carlos Rodriguez-Galindo, Catherine G. Lam, Robert E. Ruiz, Gerard P. Zambetti, Raul C. Ribeiro
Frontiers in Endocrinology.2021;[Epub] CrossRef
- Beckwith–Wiedemann syndrome: Clinical, histopathological and molecular study of two Tunisian patients and review of literature
Case Report
- Primary Leiomyosarcoma of Adrenal Gland: A Case Report.
- Heejeong Lee, Jinyoung Yoo, Seok Jin Kang, Byung Kee Kim
- Korean J Pathol. 2002;36(3):191-194.
- 1,755 View
- 19 Download
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Abstract
PDF
- Primary mesenchymal neoplasm of the adrenal gland is very rare. Recently we experienced a case of leiomyosarcoma of the adrenal gland in a 47-year-old female patient. The resected adrenal gland showed a large lobulated mass, which replaced the entire gland. The cut surface was firm and whitish gray with foci that showed hemorrhage and necrosis. Histologically, the tumor was composed of intersecting fascicles of pleomorphic spindle cells with numerous giant cells and mitotic figures. Some of the tumor cells showed elongated nuclei. Immunohistochemical studies were strongly positive for vimentin and smooth muscle actin. Cytokeratin, desmin, alpha-1-antitrypsin and lysozyme were all negative. To the best of our knowledge, this is the first case reported in Korea.
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