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埃塞俄比亚三级转诊医院治疗的儿童原发性骨肿瘤的模式和治疗结果。

Patterns and treatment outcomes of primary bone tumors in children treated at tertiary referral hospital, Ethiopia.

机构信息

Department of Pediatrics and Child Health, Debretabor University, School of Medicine, Debretabor, Ethiopia.

Department of Pediatrics and Child Health, Addis Ababa University, School of Medicine, Addis Ababa, Ethiopia.

出版信息

BMC Cancer. 2024 Mar 28;24(1):394. doi: 10.1186/s12885-024-12169-x.

Abstract

BACKGROUND

Bone tumors account for approximately 6% of all cancers in children. Malignant bone tumors, commonly occurring in children and adolescents, are associated with high mortality and morbidity. The overall survival of children with primary malignant bone tumors is affected by the stage of disease, time of diagnosis, and treatment response. Despite advanced treatment modalities with chemotherapy, surgery, and radiotherapy, bone tumor is the third leading cause of death in children with malignancy. Patients with metastatic disease at diagnosis have poor outcomes compared to localized disease at presentation. The 5-year Overall Survival and event-free survival in children with primary malignant bone tumors were 85.2% and 69.2%. The study aimed to assess the clinicopathological profile and treatment outcomes of children with primary malignant bone tumors in our setup.

MATERIALS AND METHODS

A hospital-based cross-sectional study was conducted on 95 children who met the inclusion criteria through structured questionnaire. The collected data were analyzed using a statistical package for social sciences (SPSS) version 25. P-value < 0.05 was considered to be statistically significant. Kaplan Meier survival estimate was used for overall and event-free survival analysis.

RESULTS

A total of ninety-five patients met the study inclusion criteria and the median age at diagnosis with primary malignant bone tumors was 10 years, with an interquartile range of 8-12 years. The duration of the illness from the onset of symptoms to the oncologic treatment center ranges from three weeks to 2 years with a mean duration of five months. Swelling was the commonest presenting symptom accounting for 95.8% (n = 91). Lower extremity was the commonest primary site of involvement accounting for 55.8% (n = 53) of children with primary malignant bone tumors. Osteosarcoma was the commonest malignant bone tumor constituted 66.3% (n = 63), followed by Ewing sarcoma at 33.7% (n = 32). About 41.2% (n = 39) of children had metastatic disease at presentation and the lung was the commonest site of distant metastasis. The Kaplan Meier survival estimate analysis showed the 1-year and 5-year overall survival probabilities for all pediatric primary malignant bone tumor patients were 65% (95% CI: 0.3-0.56) and 38% (95% CI:0.19-0.47) respectively. The 1-year and 5-year event-free survival probabilities were 55% (95% CI: 0.32-0.73) and 33% (95% CI: 0.10-0.59). The stage of the disease at presentation had a significant association with the outcome (p = 0.023).

CONCLUSION

Our study showed the mean duration of the illness from the onset of symptoms to the oncologic treatment center was 5 months ranging from 3 weeks to 2 years. More than one-third of the presented with metastatic disease at presentation. The 1-year and 5-year overall survival (OS) probabilities of children with primary malignant bone tumors were low in our setup compared to other studies.

摘要

背景

骨肿瘤约占儿童所有癌症的 6%。恶性骨肿瘤常见于儿童和青少年,与高死亡率和发病率有关。儿童原发性恶性骨肿瘤的总体生存率受疾病分期、诊断时间和治疗反应的影响。尽管采用化疗、手术和放疗等先进的治疗方法,但骨肿瘤仍是儿童恶性肿瘤死亡的第三大原因。与局部疾病相比,初诊时转移性疾病的患者预后较差。儿童原发性恶性骨肿瘤的 5 年总生存率和无事件生存率分别为 85.2%和 69.2%。本研究旨在评估我们机构中儿童原发性恶性骨肿瘤的临床病理特征和治疗结果。

材料和方法

通过结构化问卷对符合纳入标准的 95 名儿童进行了一项基于医院的横断面研究。使用社会科学统计软件包(SPSS)版本 25 分析收集的数据。P 值<0.05 被认为具有统计学意义。采用 Kaplan-Meier 生存估计进行总体和无事件生存分析。

结果

共有 95 名患者符合研究纳入标准,原发性恶性骨肿瘤的中位诊断年龄为 10 岁,四分位间距为 8-12 岁。从症状出现到肿瘤治疗中心的病程时间从 3 周到 2 年不等,平均病程为 5 个月。肿胀是最常见的首发症状,占 95.8%(n=91)。下肢是最常见的受累原发部位,占 55.8%(n=53)的儿童原发性恶性骨肿瘤。骨肉瘤是最常见的恶性骨肿瘤,占 66.3%(n=63),其次是尤文肉瘤,占 33.7%(n=32)。约 41.2%(n=39)的儿童在初诊时存在转移性疾病,肺部是最常见的远处转移部位。Kaplan-Meier 生存估计分析显示,所有儿科原发性恶性骨肿瘤患者的 1 年和 5 年总生存率分别为 65%(95%CI:0.3-0.56)和 38%(95%CI:0.19-0.47)。1 年和 5 年无事件生存率分别为 55%(95%CI:0.32-0.73)和 33%(95%CI:0.10-0.59)。初诊时的疾病分期与预后有显著相关性(p=0.023)。

结论

我们的研究表明,从症状出现到肿瘤治疗中心的病程时间平均为 5 个月,从 3 周到 2 年不等。超过三分之一的患者在初诊时就存在转移性疾病。与其他研究相比,我们机构中儿童原发性恶性骨肿瘤的 1 年和 5 年总生存率(OS)较低。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1223/10976724/770dd2901a75/12885_2024_12169_Fig1_HTML.jpg

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