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术后进展为中枢性性早熟的男性化肾上腺皮质癌

Virilizing adrenocortical carcinoma advancing to central precocious puberty after surgery.

作者信息

Kim Min Sun, Yang Eu Jeen, Cho Dong Hyu, Hwang Pyung Han, Lee Dae-Yeol

机构信息

Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea. ; Research Institute of Clinical Medicine of Chonbuk National University-Biomedical Research Institute, Chonbuk National University Hospital, Jeonju, Korea.

Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea.

出版信息

Korean J Fam Med. 2015 May;36(3):150-3. doi: 10.4082/kjfm.2015.36.3.150. Epub 2015 May 22.

Abstract

Adrenocortical carcinoma (ACC) in pediatric and adolescent patients is rare, and it is associated with various clinical symptoms. We introduce the case of an 8-year-old boy with ACC who presented with peripheral precocious puberty at his first visit. He displayed penis enlargement with pubic hair and facial acne. His serum adrenal androgen levels were elevated, and abdominal computed tomography revealed a right suprarenal mass. After complete surgical resection, the histological diagnosis was ACC. Two months after surgical removal of the mass, he subsequently developed central precocious puberty. He was treated with a gonadotropin-releasing hormone agonist to delay further pubertal progression. In patients with functioning ACC and surgical removal, clinical follow-up and hormonal marker examination for the secondary effects of excessive hormone secretion may be a useful option at least every 2 or 3 months after surgery.

摘要

小儿和青少年肾上腺皮质癌(ACC)较为罕见,且与多种临床症状相关。我们介绍一例8岁ACC男孩病例,其初诊时表现为外周性性早熟。他出现阴茎增大、阴毛生长和面部痤疮。其血清肾上腺雄激素水平升高,腹部计算机断层扫描显示右肾上腺肿块。完整手术切除后,组织学诊断为ACC。肿块手术切除两个月后,他随后出现中枢性性早熟。给予他促性腺激素释放激素激动剂治疗以延缓青春期进一步进展。对于功能性ACC且已行手术切除的患者,术后至少每2至3个月进行临床随访和激素标志物检查以评估激素分泌过多的继发效应可能是一种有用的选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23f8/4445056/c22b9f508bf8/kjfm-36-150-g001.jpg

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