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Pedican
Pediatric cancer database
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Basic Information

Gene ID

10984

Name

KCNQ1OT1

Synonymous

KCNQ1-AS2|KCNQ10T1|KvDMR1|KvLQT1-AS|LIT1|NCRNA00012;KCNQ1 opposite strand/antisense transcript 1 (non-protein coding);KCNQ1OT1;KCNQ1 opposite strand/antisense transcript 1 (non-protein coding)

Definition

-

Position

11p15

Gene type

miscRNA

Cancer type

Abstract

adrenocortical Tumors;Endocrine

pediatric adrenal tumors, other than neuroblastoma, are rare and can be associated with a genetic predisposition. In this report we describe two patients with an isolated and apparently sporadic adrenocortical tumor; one girl with a carcinoma, the other girl with an adenoma. In both patients genetic screening revealed hypomethylation of the KCNQ1OT1 gene, well-known for its association with the Beckwith-Wiedemann syndrome. This represents a likely novel genetic predisposition in patients with adrenocortical tumors without clear phenotypic features of the Beckwith-Wiedemann syndrome.

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