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过度生长综合征的随访与肿瘤风险。

Follow-up and risk of tumors in overgrowth syndromes.

作者信息

Gracia Bouthelier Ricardo, Lapunzina Pablo

机构信息

Department of Pediatric Endocrinology, Hospital Universitario La Paz, Autonoma University, Madrid, Spain.

出版信息

J Pediatr Endocrinol Metab. 2005 Dec;18 Suppl 1:1227-35. doi: 10.1515/jpem.2005.18.s1.1227.

Abstract

A striking feature of the overgrowth syndromes (OGS) is the risk of cancer. In some OGS (Beckwith-Wiedemann, Perlman, Simpson-Golabi-Behmel syndromes and hemihypertrophy) tumors appeared mostly in the abdomen (more than 94% of tumors), are usually diagnosed before 10 years, and most of them are embryonal. Conversely, in other OGS, such as Sotos syndrome, the most frequent type is lympho-hematological tumors, about two-thirds are extra-abdominal and some of these tumors may appear after the second decade of life. Based on a previous literature review, a specific schedule protocol for tumor screening was suggested for many OGS. In this article we briefly review some aspects of the current knowledge of OGS and tumors, emphasizing the follow-up of these disorders.

摘要

过度生长综合征(OGS)的一个显著特征是患癌风险。在某些OGS(贝克威思-维德曼综合征、佩尔曼综合征、辛普森-戈拉比-贝梅尔综合征和半侧肥大)中,肿瘤大多出现在腹部(超过94%的肿瘤),通常在10岁前被诊断出来,且大多数为胚胎性肿瘤。相反,在其他OGS中,如索托斯综合征,最常见的肿瘤类型是淋巴造血系统肿瘤,约三分之二位于腹部外,其中一些肿瘤可能在生命的第二个十年后出现。基于之前的文献综述,针对许多OGS提出了特定的肿瘤筛查时间表方案。在本文中,我们简要回顾了OGS和肿瘤的当前知识的一些方面,重点强调了对这些疾病的随访。

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