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.

     

Perinatal and familial risk factors for soft tissue sarcomas in childhood through young adulthood: A population-based assessment in 4 million live births

Perinatal factors have been associated with soft tissue sarcomas (STS) in case-control studies. However, (i) the contributions of factors including fetal growth remain unknown, ( ii ) these factors have not been examined in cohort studies and (iii) few assessments have evaluated risk in specific STS subtypes. We sought to identify the role of perinatal and familial factors on the risk of STS in a large population-based birth cohort. We identified 4,023,436 individuals in the Swedish Birth Registry born during 1973–2012. Subjects were linked to the Swedish Cancer Registry, where incident STS cases were identified. We evaluated perinatal and familial factors obtained from Statistics Sweden, including fetal growth, gestational age, and presence of a congenital malformation. Poisson regression was used to estimate incidence rate ratios (IRRs) and 95% confidence intervals (CIs) for associations between perinatal factors and STS overall, as well as by common subtypes. There were 673 individuals diagnosed with STS in 77.5 million person-years of follow-up. Having a congenital malformation was associated with STS (IRR = 1.70, 95% CI: 1.23–2.35). This association was stronger (IRR = 2.90, 95% CI: 1.25–6.71) in recent years (2000–2012). Low fetal growth was also associated with STS during the same time period (IRR = 1.86, 95% CI: 1.05–3.29). Being born preterm was associated with rhabdomyosarcoma (IRR = 1.74, 95% CI: 1.08–2.79). In our cohort study, those with congenital malformations and other adverse birth outcomes were more likely to develop a STS compared to their unaffected contemporaries. These associations may point to disrupted developmental pathways and genetic factors influencing the risk of STS.     

The use of isotretinoin for acne – an update on optimal dosing,surveillance, and adverse effects

ABSTRACT

Introduction

Acne is a chronic, inflammatory, and immune mediated disease of pilosebaceous unit, highly prevalent in adolescents. It involves face, trunk, and back; may leave scars and affect quality of life. Early, effective, and safe treatment is the key for disease resolution. Oral isotretinoin is the unique treatment for cure or prolonged remission for moderate and severe acne, preventing psychosocial impact and scars. It inhibits sebaceous glands activity and has anti-inflammatory and immunoregulatory properties.     

乌灵胶囊治疗焦虑性失眠症临床研究

目的:观察乌灵胶囊对焦虑性失眠症患者的心理及睡眠质量的改善效果,为临床治疗焦虑性失眠症提供参考。方法:选择159例焦虑性失眠症患者为研究对象。按照随机数字表法分为对照组79例与观察组80例。对照组给予盐酸帕罗西汀片治疗,观察组给予乌灵胶囊治疗。治疗1个月后,比较分析2组患者睡眠质量、汉密尔顿焦虑量表评分(HAMA)及不良反应发生情况。结果:观察组总有效率为91.25%,明显高于对照组的78.48%(P<0.05)。治疗前,2组患者HAMA评分比较,差异无统计学意义(P>0.05);治疗1、2个月后,观察组HAMA评分明显低于对照组,差异有统计学意义(P<0.05)。观察组总不良反应发生率为6.25%,明显低于对照组的18.99%(P<0.05)。结论:乌灵胶囊治疗焦虑性失眠症能明显改善患者的睡眠质量以及心理状况,值得推广应用。     

复方倍他米松注射液联合泛昔洛韦胶囊、普瑞巴林胶囊治疗带状疱疹的临床疗效观察

目的 对复方倍他米松注射液联合泛昔洛韦胶囊、普瑞巴林胶囊治疗带状疱疹的临床疗效进行观察并探讨。方法 选取2018 年1 月～2019 年12 月我院收治的118 例带状疱疹患者为研究对象，采用随机抽签的方式将患者分为对照组（泛昔洛韦胶囊+普瑞巴林胶囊+甲钴胺片+维生素B1 片）与观察组（联合复方倍他米松注射液），比较两组治疗总有效率、VAS 评分、QS 评分、C 反应蛋白、无新发皮疹时间、结痂时间及疼痛停止时间、随访2 个月后带状疱疹后遗神经痛发生率以及不良反应发生率。结果 观察组与对照组治疗总有效率分别为94.92%及76.27%，组间对比差异有统计学意义（P＜0.05）；治疗后，观察组VAS 评分显著低于对照组；观察组QS 评分低于对照组（P＜0.05）；治疗后，观察组C 反应蛋白显著低于对照组（P＜0.05）；观察组无新发皮疹时间、结痂时间及疼痛停止时间分别显著短于对照组（P＜0.05）；随访2 个月后，发现观察组带状疱疹后遗神经痛发生率显著低于对照组（P＜0.05）；观察组不良反应发生与对照组比较，差异无统计学意义（P＞0.05）。结论 复方倍他米松注射液联合泛昔洛韦胶囊、普瑞巴林胶囊治疗带状疱疹不仅效果好且安全性高，值得使用。     

针灸配合松龄血脉康胶囊治疗急性脑梗死疗效观察及对 APN、ET-1 水平的影响

目的:探究针灸配合松龄血脉康胶囊治疗急性脑梗死(ACI)的疗效及对脂联素(APN)、内皮素-1 (ET-1)水平的影响。方法:将62例ACI患者作为研究对象,按随机数字表法分成2组,每组各31例。对照组采用松龄血脉康胶囊治疗,观察组为针灸配合松龄血脉康胶囊治疗;观察2组临床疗效,并比较APN、ET-1、超敏C-反应蛋白(hs-CRP)及纤维蛋白原(FIB)。结果:观察组总有效率高达90.23%,显著高于对照组的64.52%,2组比较,差异有统计学意义(χ^2=5.905,P<0.05)。经治疗,2组治疗后APN显著升高(P<0.05),ET-1、hs-CRP、FIB均下降(P<0.05)。治疗后组间比较,观察组患者APN高于对照组(P<0.05),ET-1、hs-CRP、FIB均低于对照组(P<0.05)。结论:对ACI患者行针灸配合松龄血脉康胶囊治疗,能够显著增加APN水平,降低ET-1水平,且其炎性反应、凝血功能改善效果更为显著。     

越鞠丸联合盐酸氟西汀胶囊治疗抑郁症临床研究

目的:观察越鞠丸联合盐酸氟西汀胶囊治疗抑郁症的临床效果及对血清脑源性神经营养因子(BDNF)水平的影响。方法:纳入因抑郁症就诊的64例患者,随机分为对照组与治疗组各32例。对照组给予盐酸氟西汀胶囊抗5-羟色胺(5-HT)治疗,治疗组在对照组基础上联合越鞠丸治疗。比较2组治疗前后汉密尔顿抑郁量表(HAMD-24)评分、抑郁症状学快速自评量表(QIDS-SR16)、血清BDNF水平及总体疗效。结果:治疗前后比较,2组HAMD-24评分、QIDS-SR16评分均下降,血清BDNF水平升高(P<0.05),组间比较,治疗组2项评分下降更多,血清BDNF水平升高更多(P<0.05)。治疗后,治疗组总体疗效为90.63%,优于对照组的68.75%,差异有统计学意义(P<0.05)。结论:越鞠丸联合盐酸氟西汀胶囊治疗抑郁症,可更加有效减缓患者抑郁症状,升高血清BDNF水平,增加临床疗效,有效保护患者脑功能,值得临床推广。     

Liquid‐based cytology of pigmented phaeohyphomycotic lesion of the palm masquerading as a metastatic tumor

Phaeohyphomycosis is a chronic subcutaneous fungal infection caused by dematiaceous fungi, commonly involving the extremities, which present as single or multiple lesions mimicking a benign tumor. Fine‐needle aspiration cytology (FNAC) is an easy method for the diagnosis of the lesion. Morphology of the fungus is characteristic with no difficulty for cytopathologist for identification; with a close differential diagnosis of Candida and Aspergillosis. The special stains like periodic acid schiff (PAS) highlights the fungus and the Masson Fontana confirm the melanin pigment and dematiaceous nature of the fungi. Liquid‐based cytology (LBC) preparation of the aspirated material was attempted, which is not usually performed on day to day basis. However, in our case the conventional smears were better for appreciating the fungal morphology.