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儿童软组织肉瘤的临床表现:系统评价和荟萃分析。

Clinical presentation of childhood soft tissue sarcomas: a systematic review and meta-analysis.

机构信息

Institute of Child Health, University College London, London, UK.

Lifespan and Population Health, University of Nottingham, Nottingham, UK.

出版信息

Arch Dis Child. 2024 Jan 22;109(2):113-120. doi: 10.1136/archdischild-2023-325875.

DOI:10.1136/archdischild-2023-325875
PMID:37857451
Abstract

BACKGROUND

Time to diagnosis (TTD) of childhood soft tissue sarcoma (STS) is significantly associated with survival. This review aims to identify pre-diagnostic symptoms/signs to inform earlier diagnosis interventions.

METHODS

Medline, Embase, Cochrane and Web-of-Science were searched between January 2010 and February 2021 for studies including children (<18 years) diagnosed with STS, with no language restrictions. Pooled proportions of symptoms/signs were calculated and subanalysed by tumour location and age.

RESULTS

Fifty-nine eligible studies were identified, totalling 2462 cases. The most frequent symptoms were lump/swelling (38%, 95% CI 27% to 51%), pain (6%, 95% CI 3% to 10%), cutaneous changes (4%, 95% CI 0 to 9%), localised eye swelling (3%, 95% CI 0 to 7%), cranial nerve deficits (2%, 95% CI 0 to 5%) and constitutional symptoms (2%, 95% CI 0 to 5%).Symptoms varied by location and age. Localised eye swelling (20%, 95% CI 3% to 45%), cranial nerve deficits (14%, 95% CI 4% to 28%) and impaired visual function (6%, 95% CI 0 to 17%) were frequent in head and neck tumours. For abdomen/pelvic tumours, urinary symptoms (24%, 95% CI 5% to 15%), abdominal distension/discomfort (22%, 95% CI 4% to 47%), genital lump/swelling (16%, 95% CI 1% to 42%), constitutional symptoms (9%, 95% CI 0%] to 23%), vaginal bleeding (7%, 95%C I 0 to 21%) and bowel habit changes (6%, 95% CI 0 to 17%) were frequent.In <5 years, consumptive coagulopathy (16%, 95% CI 0 to 48%), cutaneous changes (5%, 95% CI 0 to 40%), genital lump/swelling (4%, 95% CI 0 to 14%), reduced mobility (3%, 95% CI 0 to 11%), vaginal bleeding (2%, 95% CI 0 to 11%) and bleeding/bruising/petechiae (2%, 95% CI 0 to 20%) were frequent compared with lump/swelling, constitutional symptoms, pain and headaches which were frequent among >11 years.

CONCLUSIONS

For STS, pre-diagnostic symptoms differ by age and location, highlighting the need to tailor early diagnosis interventions.

摘要

背景

儿童软组织肉瘤(STS)的诊断时间(TTD)与生存显著相关。本综述旨在确定诊断前的症状/体征,以告知更早的诊断干预措施。

方法

在 2010 年 1 月至 2021 年 2 月期间,检索了 Medline、Embase、Cochrane 和 Web-of-Science,纳入了诊断为 STS 的儿童(<18 岁)的研究,无语言限制。计算了症状/体征的合并比例,并按肿瘤位置和年龄进行了亚分析。

结果

确定了 59 项符合条件的研究,共纳入 2462 例病例。最常见的症状是肿块/肿胀(38%,95%CI 27%至 51%)、疼痛(6%,95%CI 3%至 10%)、皮肤改变(4%,95%CI 0 至 9%)、局部眼部肿胀(3%,95%CI 0 至 7%)、颅神经缺陷(2%,95%CI 0 至 5%)和全身症状(2%,95%CI 0 至 5%)。症状因位置和年龄而异。局部眼部肿胀(20%,95%CI 3%至 45%)、颅神经缺陷(14%,95%CI 4%至 28%)和视力受损(6%,95%CI 0 至 17%)在头颈部肿瘤中较为常见。对于腹部/盆腔肿瘤,泌尿系统症状(24%,95%CI 5%至 15%)、腹部膨胀/不适(22%,95%CI 4%至 47%)、生殖器肿块/肿胀(16%,95%CI 1%至 42%)、全身症状(9%,95%CI 0%至 23%)、阴道出血(7%,95%C I 0 至 21%)和肠道习惯改变(6%,95%CI 0 至 17%)较为常见。在<5 岁的患者中,消耗性凝血病(16%,95%CI 0 至 48%)、皮肤改变(5%,95%CI 0 至 40%)、生殖器肿块/肿胀(4%,95%CI 0 至 14%)、活动能力下降(3%,95%CI 0 至 11%)、阴道出血(2%,95%CI 0 至 11%)和出血/瘀伤/瘀点(2%,95%CI 0 至 20%)较常见,而肿块/肿胀、全身症状、疼痛和头痛在>11 岁的患者中更为常见。

结论

对于 STS,诊断前的症状因年龄和位置而异,这突出表明需要针对早期诊断干预措施进行调整。

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