Metastatic adult-type non-rhabdomyosarcoma soft tissue sarcomas in children and adolescents: A cohort study from the European paediatric Soft tissue sarcoma Study Group

Background

Limited data exist on the clinical behavior of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with distant metastases at onset, and a clear standard of care has not yet been defined.

Methods

This cohort study reports on pediatric adult-type metastatic NRSTS enrolled in two concurrent prospective European studies, i.e., the randomized BERNIE study and the single-arm MTS 2008 study developed by the European paediatric Soft tissue sarcoma Study Group. Treatment programs were originally designed for patients with metastatic rhabdomyosarcoma, i.e., nine courses of multidrug chemotherapy (with or without bevacizumab in the BERNIE study), followed by 12 cycles of maintenance therapy, whereas radiotherapy and/or surgery (on primary tumor and/or metastases) were delayed until after seven courses of chemotherapy had been administered.

Results

The study included 61 patients <21 years old treated from July 2008 to December 2016. The lung was the site of metastases in 75% of the cases. All patients received multi-agent chemotherapy, 44% had local therapy to primary tumor, and 18% had treatment of metastases. Median time to progression/relapse was 6 months. A high rate of tumor progression was observed during the initial part of the chemotherapy program. With a median follow-up of 41.5 months (range, 2–111 months), 3-year event-free survival and overall survival were 15.4% (95% confidence interval [CI], 7.6–25.7) and 34.9% (95% CI, 22.7–47.5), respectively. There were no statistically significant differences in outcome depending on the type of treatment administered.

Conclusions

The study confirmed the overall poor outcome for patients with metastatic NRSTS, whose treatment remains a challenge.

Plain Language Summary

• Pediatric non-rhabdomyosarcoma soft tissue sarcomas form a heterogeneous group of rare tumors.
• Although recent international studies have defined the standard of care for patients with localized disease, limited data are available on the clinical behavior of patients with distant metastases.
• This study on 61 metastatic cases treated on two prospective European protocols confirms that the chances of survival of such patients are often dismal and a standard treatment is still lacking.

     

原发灶不明骨髓转移癌25例临床分析北大核心

目的:探讨原发灶不明骨髓转移癌的病例特点、治疗情况及预后。方法:对纳入的25例患者进行回顾性分析其各自的临床特点、治疗过程以及生存情况。结果:25例患者中位总生存期为6个月。初诊时是否伴有血液学症状、病理类型、治疗方式以及接受治疗时间对患者生存有显著差异。治疗方式、家庭支持强度是影响预后的独立因素。结论:原发灶不明骨髓转移癌是临床少见的疾病,给予积极的综合治疗,加强社会家庭的支持强度可能会改善其预后。     

颞下颌关节骨关节病术后不同辅助治疗效果评价

目的    评价颞下颌关节骨关节病（temporomandibular joint osteoarthrosis，TMJOA）术后不同辅助治疗方法的临床效果，为术后康复治疗提供参考。方法    选取2015年6月至2021年11月于中国医科大学附属口腔医院口腔颌面外科行颞下颌关节盘松解复位固定手术的191例TMJOA患者进行回顾性分析。术后行牙合垫治疗+常规功能训练的63例患者记为牙合垫组，行几丁糖关节腔内注射治疗+常规功能训练的57例患者记为注射组，仅行常规功能训练的71例患者记为对照组。对3组患者治疗前后的疼痛视觉模拟评分法（VAS）评分、最大张口度、下颌运动分、肌肉压诊分进行比较分析。结果    治疗前，3组各项评价指标比较，差异均无统计学意义（均P > 0.05）。治疗后3个月，3组各项评价指标总的比较，差异均有统计学意义（均P < 0.05）；关于VAS评分、下颌运动分、肌肉压诊分方面，分值由低至高依次为注射组、牙合垫组、对照组，而最大张口度由大至小依次为注射组、牙合垫组、对照组，差异均有统计学意义（P < 0.05）；且3组各项评价指标结果均明显优于治疗前（均P < 0.05）。结论    TMJOA患者行颞下颌关节盘松解复位固定手术后采取适当辅助治疗和功能训练均能显著改善颞下颌关节功能，其中几丁糖关节腔内注射治疗结合常规功能训练的效果较佳。     

外泌体在卵巢癌诊疗及转移中的研究进展

卵巢癌是妇科肿瘤领域内的一个热点主题。该种恶性肿瘤疾病的发病率高且隐匿,缺乏有效的早期发现及时诊断并治疗的方式,病情容易加重发生远处转移。因此罹患卵巢癌的女性5年生存率比较低。外泌体属于一类典型的囊性泡样的小体,参与肿瘤的发病及转移机制且在肿瘤疾病的治疗中起着重要作用。本篇综述主要介绍癌细胞外泌体在卵巢恶性肿瘤疾病的诊断、转移及治疗中的研究现状。     

Reproduction patterns among classical Hodgkin lymphoma survivors treated with BEACOPP and ABVD in Sweden,Denmark and Norway—A population-based matched cohort study

Childbirth rates in classical Hodgkin lymphoma (cHL) survivors have historically been reduced compared to the general population. Understanding if contemporary treatment protocols are associated with reduced fertility is crucial as treatment guidelines shift toward more liberal use of intensive chemotherapy. We identified 2834 individuals aged 18-40 years with cHL in Swedish and Danish lymphoma registers, and in the clinical database at Oslo University Hospital diagnosed 1995-2018, who were linked to national medical birth registers. Cox regression adjusted for stage, performance status, year, and age at diagnosis was used to estimate hazard ratios (HRs) and 95% confidence intervals (CI) contrasting time to first childbirth by treatment groups (ABVD, 2-4 BEACOPP, 6-8 BEACOPP) up to 10 years after diagnosis. Overall, 74.8% of patients were treated with ABVD, 3.1% with 2-4 BEACOPP and 11.2% with 6-8 BEACOPP. Adjusted HRs comparing childbirth rates in individuals treated with 6-8 BEACOPP, and 2-4 BEACOPP to ABVD were 0.53 (CI: 0.36-0.77) and 0.33 (CI: 0.12-0.91) for males, and 0.91 (CI: 0.61-1.34) and 0.38 (CI: 0.12-1.21) for females. Cumulative incidence of childbirths after 10 years was 19.8% (CI: 14.5%-27.0%) for males and 34.3% (CI: 25.8%-45.6%) for females treated with 6-8 BEACOPP. Proportions of children born after assisted reproductive technique (ART) treatments were 77.4% (CI: 60.2-88.6%) for males following 6-8 BEACOPP, and <11% for females. Among ABVD treated patients the corresponding proportions were 12.2% (CI: 8.5%-17.3%) and 10.6% (CI: 7.4%-14.9%). BEACOPP treatment is associated with decreased childbirth rates compared to ABVD in male, but not female, cHL patients, despite widespread access to ART in the Nordics.     

基于CNKI的中医药治疗癫痫的知识图谱分析＊

目的 运用科学知识图谱的手段，分析中医药治疗癫痫的研究进程并提炼重点。方法 设置检索时间为1990年至2020年，检索对象为中国知网（CNKI）该领域的相关文献，运用CiteSpace软件科学、准确地分析这些文献的作者信息、研究（发文）机构、关键词情况。结果 共计纳入文献793篇，马融-张喜莲、刘金民、谢炜、王洪图-张丽萍等领导核心作者团队，北京、广州以及广西中医药大学、天津中医药大学第一附属医院、南方医科大学等单位为主要进行相关研究的机构，高频关键词包括大鼠、难治性癫痫、小儿癫痫、海马、脑电图等，作用机制、网络药理学、肠道菌群、氧化应激、分子生物学等为近年来的热点与前沿。结论 通过借用知识图谱的分析能帮助提炼国内中医药治疗癫痫研究领域的重点，全面分析并科学判断近年来该领域的热点及前沿，为未来的研究夯实基础并把握方向。     

肝移植术后移植物抗宿主病的诊治进展

肝移植术后移植物抗宿主病(GVHD)发生率低，但病死率极高，典型症状包括不明原因发热、皮疹、腹泻和骨髓抑制。其发病机制目前尚未完全阐明，诊断缺乏统一标准，治疗缺乏特异性手段。近年来，针对GVHD免疫反应进程中的病理生理学研究，为开辟新的诊断方法和治疗手段提供可能。目前，大剂量糖皮质激素联合静脉注射免疫球蛋白冲击、免疫抑制剂减少或停用可能是较为有效的治疗方案。而对于糖皮质激素难治性GVHD，应用多种不同的分子靶向药物可能对改善受者预后起到积极作用。