CNC心脏黏液瘤的研究进展

Carney综合征（carney complex,CNC）是一种因17号染色体基因突变导致的累及全身多器官系统的罕见疾病,具有家族聚集性的特点。临床表现以皮肤改变、心脏肿瘤等多见。本文围绕CNC心脏黏液瘤的发病特点,说明散发性心脏黏液瘤与CNC心脏肿瘤的不同,阐述CNC心脏黏液瘤临床表现并结合超声心动图等多种辅助检查明确心脏黏液瘤的诊断优势,说明CNC的诊断标准,为临床诊断和治疗提供方向。心脏黏液瘤治疗方法目前主要包括:胸骨正中切开术、微创等,介绍两种术式利弊,为临床治疗提供参考。本文针对CNC累及心脏的临床表现、辅助检查、治疗等进行概述,以期为临床医生提高诊断率和选择合适治疗方案提供思路。     

卡尼复合征心脏黏液瘤的临床特征及外科治疗结果

目的  比较卡尼复合征（Carney complex）心脏黏液瘤与散发心脏黏液瘤的临床特征及外科治疗结果,为Carney complex心脏黏液瘤提供诊疗经验。 方法 回顾性分析阜外医院2009年1月至2013年12月共372例接受心脏黏液瘤切除手术患者的临床资料,其中7例确诊为Carney complex心脏黏液瘤。7例中男2例,女5例;年龄7～50岁,平均（2857±1326）岁;均接受外科手术切除。术后随访54～94个月,平均（7686±1549）个月。比较Carney complex心脏黏液瘤和散发心脏黏液瘤在年龄、性别、术前动脉栓塞率、心脏黏液瘤多发性和心脏黏液瘤切除术后的复发率。 结果 手术切除Carney complex心脏黏液瘤在总体心脏黏液瘤中的发病率为188％,最常见的发生部位是左房。所有7例Carney complex心脏黏液瘤术后没有早期死亡,随访期间无死亡。Carney complex心脏黏液瘤与散发性心脏黏液瘤在年龄、术前动脉栓塞率、心脏黏液瘤多发率及心脏黏液瘤术后复发率的差异均有统计学意义（P＜005）,而在性别上差异无统计学意义（P＞005）。 结论  Carney complex心脏黏液瘤可取得良好的外科疗效。与散发心脏黏液瘤相比,Carney complex心脏黏液瘤发病年龄更早,术前动脉栓塞率更高,常见多发肿瘤,术后复发率更高。对于Carney complex心脏黏液瘤,外科手术应更积极,术后应更密切随访。     

心脏黏液瘤176例的诊断与外科治疗临床分析

目的：总结176例心脏黏液瘤外科治疗经验。方法：统计我院手术治疗176倒心脏黏液瘤患者临床资料。结果：左心房黏液瘤160例，其中心脏多发瘤6例；右心房瘤12例；双房瘤3例；左心室瘤1例。合并二尖瓣关闭不全5例。均由超声心动图确诊，并在全麻体外循环下手术摘除黏液瘤，同期行二尖辩成形和二尖瓣替换术5例。术后5例（2．84％）死亡。随访9个月～16年，6例（3．4％）复发。结论：超声心动图对心脏黏液瘤的确诊具有特殊价值，立即彻底切除是预防复发的关键。     

心脏黏液瘤48例外科治疗

 目的　探讨心脏黏液瘤的临床诊断与外科手术治疗的特点。方法　1997年1月至2007年12月收治的48例心脏黏液瘤患者在体外循环下接受手术治疗，同期施行三尖瓣成形术5例，房间隔缺损修补术6例，二尖瓣成形术2例，二尖瓣替换术1例。结果　所有患者术前均经彩色超声明确诊断。全部患者在体外循环下切除肿瘤，无死亡病例。其中左心房黏液瘤42例，右心房黏液瘤6例。经病理证实全部为黏液瘤。共获随访22例，2例黏液瘤患者术后12个月复发。结论　彩色超声心动图对心脏黏液瘤的确诊具有特殊价值，心脏黏液瘤有阻碍血流、栓塞和猝死的危险，一经确诊应尽快手术切除。     

单纯型和混合型乳腺黏液腺癌的超声特征分析

目的 分析单纯型乳腺黏液腺癌（pure mucinous breast carcinoma,PMBC）和混合型乳腺黏液腺癌（mixed mucinous breast carcinoma,MMBC）的二维超声和彩色多普勒血流显像特征。方法 回顾性分析2013年1月至2020年12月于我院就诊且经手术病理证实的49例乳腺黏液腺癌（mucinous breast carcinoma,MBC）患者的二维超声、彩色多普勒血流显像表现,比较PMBC和MMBC患者在形态、边缘、内部回声、后方回声、钙化、血流分级和BI-RADS分类等方面的差异。结果 49例MBC病灶中,BI-RADS 3类1例,BI-RADS 4a类4例,BI-RADS 4b类15例,BI-RADS 4c类11例,BI-RADS 5类18例。PMBC组肿块呈混合回声的显示率高于MMBC组（P=0.025）;MMBC组肿块后方回声衰减及边缘毛刺、模糊的显示率高于PMBC组（P=0.008,0.023）;MMBC组血流3级肿块和BI-RADS 5类肿块占比均高于PMBC组;BI-RADS 4b类肿块占比低于PMBC组。结论 PMBC较...     

47例心脏黏液瘤的诊治分析

目的　探讨心脏黏液瘤诊治的经验 ,减少误诊。方法　对 47例心脏黏液瘤患者诊疗确诊后进行全麻体外循环下心内直视手术摘除 ,分析、总结。结果　许多心脏黏液瘤患者症状不典型 ,容易误诊。结论　对怀疑心脏黏液瘤的患者应及早作心脏超声确诊 ,尽早手术 ,减少栓塞 ,猝死等并发症。     

心脏黏液瘤的研究和诊疗进展

心脏黏液瘤是最为常见的心脏良性肿瘤,危害较大.临床表现缺乏特异性,诊断存在一定困难.近年来,对于心脏黏液瘤的相关临床和基础研究有所进展,本文对心脏黏液瘤的流行病学、分类、相关检查、诊断、相关基础研究及相关治疗、预后等方面的研究和诊疗进展进行总结.     

47例心脏黏液瘤的诊治分析

目的 探讨心脏黏液瘤诊治的经验，减少误诊。方法 对47例心脏黏液瘤患者诊疗确诊后进行全麻体外循环下心内直视手术摘除，分析、总结。结果 许多心脏黏液瘤患者症状不典型，容易误诊。结论 对怀疑心脏黏液瘤的患者应及早作心脏超声确诊，尽早手术，减少栓塞，猝死等并发症。     

心脏黏液瘤患者心功能分级和栓塞发生情况及其与凝血功能的相关性分析

目的:分析心脏黏液瘤患者心功能分级和栓塞发生情况及其与凝血功能的相关性。方法:采用回顾性研究,收集本院83例心脏黏液瘤患者的临床资料,记录患者主要临床症状,比较不同心功能分级的患者肿瘤最大径、血小板计数（PLT）、左心室射血分数（LVEF）、凝血酶原时间（PT）及纤维蛋白原（Fib）的差异,并比较发生下肢血管或脑栓塞患者与无栓塞患者肿瘤最大径、PLT、LVEF、PT及Fib的差异。结果:83例患者中,发生胸闷/胸痛18例（21.69%）,心悸/气促72例（86.75%）,下肢水肿13例（15.66%）,全身乏力17例（20.48%）;心脏听诊可闻及收缩期杂音22例（26.51%）,闻及舒张期杂音29例（34.94%）,双期杂音7例（8.43%）;心功能分级以Ⅱ级为主,其中Ⅰ级11例（13.25%）,Ⅱ级60例（72.29%）,Ⅲ级8例（9.64%）,Ⅳ级4例（4.82%）。超声心动图检查提示左心室黏液瘤9例（10.84%）,右心房黏液瘤7例（8.43%）,左心房黏液瘤71例（85.54%）,心包积液10例（12.05%）。心电图检查提示窦性心律不齐5例（6.02%）,ST-T段改变19例（22.89%）,窦性心动过速7例（8.43%）,左心房肥大2例（2.41%）,右心房肥大1例（1.20%）,心房颤动2例（2.41%）。随着心功能分级的升高,患者PT、Fib及PLT随之增高（P＜0.05）,而不同心功能分级患者肿瘤最大径和LVEF比较,并无明显差异（P＞0.05）。83例患者中,9例（10.84%）出现下肢血管或脑栓塞,此类患者PT、Fib及PLT较无栓塞者明显升高（P＜0.05）,而肿瘤最大径和LVEF与无栓塞者比较,并无明显差异（P＞0.05）。结论:心脏黏液瘤患者心功能分级以Ⅱ级为主,部分患者可出现下肢血管或脑栓塞,且患者心功能分级与栓塞的发生与凝血功能关系密切。     

腹壁韧带样瘤的临床与超声分析

为了探讨二维超声及彩色多普勒血流显像(CDFI)对腹壁韧带样瘤的诊断价值,对27例经病理确诊的腹壁韧带样瘤的二维声像图及彩色多普勒血流显像进行分析。27例患者中26例单发,1例多发有2个肿块。肿块最大径2.5~13cm,平均7.3cm,肿块长轴与累及的肌肉、肌腱方向一致;肿块边缘规则,与肌肉分界清晰12例,模糊不清15例。CDFI检查19例肿块内仅见稀疏点状或短棒状血流信号,8例可见条状或较丰富血流信号。初步研究结果提示,二维超声及彩色多普勒血流显像对腹壁韧带样瘤的诊断及鉴别诊断具有一定的临床价值。     

Experience with Impedance Phlebography Compared with Venography

Venography is a particularly reliable method for the diagnosis of deep venous thrombosis but is not suitable as a screening test. Impedance phlebography represents another attempt to discover a simple, non-invasive and reliable method of detecting deep venous thrombosis. It does not, however, meet these criteria.     

Compliance with guidelines and correlation with outcome in patients with advanced germ-cell tumours

PurposeTo evaluate prior compliance with guidelines in patients treated with salvage chemotherapy for advanced germ-cell tumours (GCT).Patients and methodsData concerning the initial management of patients requiring salvage chemotherapy for GCT at Institut Gustave Roussy between 2000 and 2010 were obtained and correlated with recommendations for treatment. Criteria of non-compliance were defined based on guidelines. Compliance with guidelines, predictive factors for non-compliance and the impact on outcome were analysed.ResultsAmong 82 patients treated in the salvage setting, guidelines to initial treatment were followed in only 41 cases (50%). The most common non-compliance criteria were non-adherence to the planned dose (16%), an inappropriate interval between first-line chemotherapy cycles (16%), the lack of post-chemotherapy surgery (16%) and a long interval to post-chemotherapy surgery (48%). Compliance with standard care was better in cancer centres than in other hospitals (private or public) (Odd Ratio (OR): 6.9, P = 0.001). A poor-risk status according to the International Germ Cell Cancer Collaborative Group (IGCCCG) was also predictive of compliance in univariate but not in multivariate analysis. No significant difference in outcome after salvage chemotherapy was observed. Patients relapsing after non-compliant first-line therapy tended to be more easily salvaged, which is consistent with the fact that their initial treatment was inadequate. Some of these relapses were therefore probably not due to true biologically refractory disease.ConclusionGuidelines for first-line treatment are adhered to in only half the patients requiring salvage chemotherapy. As the only predictive factor for non-compliance was the treating centre, centralisation of patients with GCT in well-trained hospitals should be recommended.     

Treatment with methylnaltrexone is associated with increased survival in patients with advanced cancer

BackgroundMethylnaltrexone (MNTX), a peripherally acting μ-opioid receptor (MOR) antagonist, is FDA-approved for treatment of opioid-induced constipation (OIC). Preclinical data suggest that MOR activation can play a role in cancer progression and can be a target for anticancer therapy.Patients and methodsPooled data from advanced end-stage cancer patients with OIC, despite laxatives, treated in two randomized (phase III and IV), placebo-controlled trials with MNTX were analyzed for overall survival (OS) in an unplanned post hoc analysis. MNTX or placebo was given subcutaneously during the double-blinded phase, which was followed by the open-label phase, allowing MNTX treatment irrespective of initial randomization.ResultsIn two randomized, controlled trials, 229 cancer patients were randomized to MNTX (117, 51%) or placebo (112, 49%). Distribution of patients' characteristics and major tumor types did not significantly differ between arms. Treatment with MNTX compared with placebo [76 days, 95% confidence interval (CI) 43–109 versus 56 days, 95% CI 43–69; P = 0.033] and response (laxation) to treatment compared with no response (118 days, 95% CI 59–177 versus 55 days, 95% CI 40–70; P < 0.001) had a longer median OS, despite 56 (50%) of 112 patients ultimately crossing over from placebo to MNTX. Multivariable analysis demonstrated that response to therapy [hazard ratio (HR) 0.47, 95% CI 0.29–0.76; P = 0.002) and albumin ≥3.5 (HR 0.46, 95% CI 0.30–0.69; P < 0.001) were independent prognostic factors for increased OS. Of interest, there was no difference in OS between MNTX and placebo in 134 patients with advanced illness other than cancer treated in these randomized studies (P = 0.88).ConclusionThis unplanned post hoc analysis of two randomized trials demonstrates that treatment with MNTX and, even more so, response to MNTX are associated with increased OS, which supports the preclinical hypothesis that MOR can play a role in cancer progression. Targeting MOR with MNTX warrants further investigation in cancer therapy.Clinical trials numberNCT00401362, NCT00672477.     

Costs associated with complications are lower with capecitabine than with 5‐fluorouracil in patients with colorectal cancer

BACKGROUND:

Capecitabine, an oral alternative to 5‐fluorouracil (5‐FU) in patients with colorectal cancer (CRC), has equal clinical efficacy and a favorable safety profile; however, its use may be limited because of unit cost concerns. In this study, the authors measured the cost of chemotherapy‐related complications during treatment with capecitabine‐ and 5‐FU–based regimens.

METHODS:

Patients with CRC who received at least 1 administration of capecitabine or 5‐FU during 2004 and 2005 were identified from the Thomson MarketScan research databases. Monthly frequency and cost for 23 complications were recorded. Logistic regression was used to predict complication probability. General linear models were used to predict monthly complication cost and total monthly expenditure.

RESULTS:

In total, 4973 patients with CRC met the inclusion criteria for this analysis. Although the most frequently observed complications were the same between capecitabine and 5‐FU (nausea and vomiting, infection, anemia, neutropenia, diarrhea), each was observed with greater frequency in 5‐FU–based regimens. The mean predicted monthly complication cost was significantly higher (by 136%) with 5‐FU monotherapy than with capecitabine monotherapy (difference, $601; 95% confidence interval [95% CI], $469‐$737). In addition, the mean predicted monthly complication cost for 5‐FU+oxaliplatin was higher than the cost with capecitabine plus oxaliplatin (difference, $1165; 95% CI, $892‐$1595). When acquisition, administration, and complication costs were taken into consideration, there were no significant differences in the total cost between capecitabine regimens and 5‐FU regimens.

CONCLUSIONS:

Capecitabine compared well with 5‐FU–based therapy in patients with CRC and was associated with lower complication rates and associated costs. Cancer 2009. © 2009 American Cancer Society.     

Living with secondary breast cancer: coping with an uncertain future with unmet needs

JOHNSTON S.R.D. (2010) European Journal of Cancer Care 19 , 561–563 Living with secondary breast cancer: coping with an uncertain future with unmet needs     

奥沙利铂联合羟基喜树碱治疗晚期胃癌临床分析

背景与目的体外及体内的临床研究显示,奥沙利铂(L-OHP)对多种肿瘤有显著抑制作用并与绝大多数抗癌药物具有相加或协同细胞毒作用.本文旨在观察L-OHP联合羟基喜树碱(HCPT)治疗晚期胃癌的近期疗效和患者耐受性,并与传统的化疗方案进行对比.方法采用非随机的分组方法将43例晚期胃癌患者分为L-OHP+HCPT方案组(治疗组)与Vp-16+CF+5-FU(ELF)方案组(对照组),其中男性28例,女性15例,中位年龄59岁,KPS评分≥60,观察两组的近期疗效和患者耐受性.结果治疗组24例有效率58.3%(14/24),对照组19例有效率42.1%(8/19).治疗组有效率高于对照组,两组差异有显著性(P<0.05).两组不良反应主要是骨髓抑制、恶心、呕吐、口腔炎、周围神经炎、静脉炎、脱发等,均在Ⅰ、Ⅱ度范围内.结论L-OHP联合HCPT方案治疗晚期胃癌疗效较好,不良反应可以耐受.     

Varicella-Zoster Virus Prophylaxis with Low-Dose Acyclovir in Patients with Multiple Myeloma Treated with Bortezomib

BackgroundVaricella-zoster virus (VZV) reactivation is a common complication in patients with multiple myeloma (MM) treated with bortezomib, with an incidence rate of 10%-60%. The aim of our study was to analyze the effect of acyclovir prophylaxis in this patient population.Patients and MethodsWe studied 98 consecutive patients with relapsed MM treated with bortezomib. Bortezomib 1.3 mg/m² was given on days 1, 4, 8, and 11 of a 21-day cycle. At first, patients did not receive any VZV prophylaxis, but because of the high incidence of VZV reactivation, VZV prophylaxis with acyclovir was implemented subsequently.ResultsA total of 11 patients treated with bortezomib did not have any VZV prophylaxis, and 4 of these 11 patients (36%) developed VZV reactivation in the form of herpes zoster. No VZV reactivations were observed in the 32 patients who received acyclovir 400 mg 3 times daily or the 55 patients who received acyclovir in a dose reduced to 400 mg once daily during bortezomib treatment.ConclusionVaricellazoster virus reactivation is a common and serious adverse effect of bortezomib treatment. Acyclovir 400 mg once daily is sufficient to protect from VZV reactivation in patients with MM treated with bortezomib.