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侧方过度生长作为小儿矫形外科医生腹部肿瘤的指导征象。

Lateralized overgrowth as a guiding sign of abdominal neoplasms for pediatric orthopedic surgeons.

机构信息

Hospital Sant Joan de Deu, Sant Joan de Déu 2, 08950 Esplugues de Llobregat, Spain.

出版信息

Jt Dis Relat Surg. 2023;34(1):3-8. doi: 10.52312/jdrs.2023.903. Epub 2023 Jan 14.

Abstract

OBJECTIVES

This study aims to increase the awareness of the association between lateralized overgrowth (LO) and abdominal tumor among the pediatric orthopedic community and to evaluate its incidence in our center.

PATIENTS AND METHODS

Between January 1997 and December 2021, a total of 166 patients with Wilms tumors and hepatoblastomas were retrospectively analyzed. Data including age, sex, initial clinical signs (hematuria, abdominal mass with or without general discomfort), type of asymmetric regional body overgrowth (isolated or in relation with any syndrome), and tumor stage at diagnosis were recorded. In addition, age at which asymmetric regional body overgrowth was described and age at the time of tumor diagnosis were noted.

RESULTS

Of a total of 166 patients, 133 were diagnosed with Wilms tumors (nephroblastomas) and 33 were diagnosed with hepatoblastomas. In 94% of the cases, the initial clinical signs were an abdominal mass and/or hematuria. Overall, five (3%) patients presented with LO. Four patients with Wilms tumor presented it at the initial clinical examinations. In three of these cases (2.3%), we found it isolated and, in the remaining patient (0.75%), it was associated with Beckwith-Wiedemann spectrum. Only one patient affected from hepatoblastoma (3%) presented with an isolated LO at the time of tumor diagnosis.

CONCLUSION

Our study results show an incidence of LO in relation to intra-abdominal tumors of 3%. The latest updates recommend genetic testing to identify subgroups with a higher risk for tumor development that are more likely to benefit from tumor protocol surveillance.

摘要

目的

本研究旨在提高儿科骨科领域对侧偏生长(LO)与腹部肿瘤之间关联的认识,并评估本中心的发病率。

患者和方法

1997 年 1 月至 2021 年 12 月,回顾性分析了 166 例患有肾母细胞瘤和肝母细胞瘤的患者。记录的数据包括年龄、性别、首发临床症状(血尿、腹部肿块伴或不伴全身不适)、不对称区域性身体生长类型(孤立或与任何综合征相关)以及诊断时的肿瘤分期。此外,还记录了描述不对称区域性身体生长的年龄和诊断肿瘤时的年龄。

结果

在总共 166 例患者中,133 例诊断为肾母细胞瘤(肾胚细胞瘤),33 例诊断为肝母细胞瘤。94%的病例首发临床症状为腹部肿块和/或血尿。总体而言,有 5 例(3%)患者出现 LO。4 例肾母细胞瘤患者在首次临床检查时出现 LO。在这 3 例患者中(2.3%),LO 是孤立的,而在其余 1 例患者中(0.75%),LO 与 Beckwith-Wiedemann 综合征有关。仅有 1 例肝母细胞瘤(3%)患者在诊断肿瘤时出现孤立性 LO。

结论

我们的研究结果显示,与腹内肿瘤相关的 LO 发病率为 3%。最新的更新建议进行基因检测,以确定肿瘤发生风险较高的亚组,这些亚组更有可能受益于肿瘤方案监测。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5054/9903098/ade8391646b3/JDRS-2023-34-1-003-008-F1.jpg

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