罕见的儿童Li-Fraumeni综合征病例,磁共振成像和[18F]-氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描显示为男性化症状和双重原发性恶性肿瘤。

IF 2.3 3区 医学 Q2 PEDIATRICS
Jiawen Chen, Wei Ouyang
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引用次数: 0

摘要

这个病例报告描述了一个19个月大的男孩,表现为阴茎大,身材增高,声音低沉,被诊断为右侧侧脑室并发脉络膜丛癌和左侧肾上腺皮质癌。随后的种系检测显示致病性TP53突变,证实了Li-Fraumeni综合征的诊断。Li-Fraumeni综合征的主要表现是大阴茎和加速线状生长,在医学文献中很少有文献记载。该病例强调了在患有生长发育异常的儿童患者,特别是那些提示Li-Fraumeni综合征的儿童患者中实施全身成像模式的重要性,以指导治疗决策和随访监测。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A rare pediatric case of Li-Fraumeni syndrome presenting with virilization symptoms and dual primary malignancies on magnetic resonance imaging and [18F]-fluorodeoxyglucose positron emission tomography/computed tomography.

This case report describes a 19-month-old boy presenting with macropenis, increased stature, and deep voice, who was diagnosed with concurrent choroid plexus carcinoma in the right lateral ventricle and left adrenocortical carcinoma. Subsequent germline testing revealed pathogenic TP53 mutations, confirming a diagnosis of Li-Fraumeni syndrome. Li-Fraumeni syndrome with primary manifestations of macropenis and accelerated linear growth has rarely been documented in the medical literature. This case highlights the critical importance of implementing whole-body imaging modalities in pediatric patients with growth-developmental abnormalities, particularly those suggestive of Li-Fraumeni syndrome, to guide therapeutic decision-making and follow-up monitoring.

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来源期刊
Pediatric Radiology
Pediatric Radiology 医学-核医学
CiteScore
4.40
自引率
17.40%
发文量
300
审稿时长
3-6 weeks
期刊介绍: Official Journal of the European Society of Pediatric Radiology, the Society for Pediatric Radiology and the Asian and Oceanic Society for Pediatric Radiology Pediatric Radiology informs its readers of new findings and progress in all areas of pediatric imaging and in related fields. This is achieved by a blend of original papers, complemented by reviews that set out the present state of knowledge in a particular area of the specialty or summarize specific topics in which discussion has led to clear conclusions. Advances in technology, methodology, apparatus and auxiliary equipment are presented, and modifications of standard techniques are described. Manuscripts submitted for publication must contain a statement to the effect that all human studies have been reviewed by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in an appropriate version of the 1964 Declaration of Helsinki. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study. Details that might disclose the identity of the subjects under study should be omitted.
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