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非肌无力性胸腺瘤切除术后临床明显重症肌无力的发生和演变

已有 2922 次阅读2023-10-10 12:21 |个人分类:TET学习|系统分类:医学科学| 胸腺瘤, 重症肌无力

Onset and Evolution of Clinically Apparent Myasthenia Gravis After Resection of Non-myasthenic Thymomas

患有胸腺瘤且无临床或肌电图肌无力症状的患者可能在胸腺切除术后数年偶尔发展为肌无力。在此，我们研究了这种特殊疾病的预测因素和演变过程。

我们对1987年至2013年间因胸腺瘤接受胸腺切除术的104名连续患者进行了回顾性分析，这些患者没有重症肌无力的临床或肌电图体征。采用单变量发病时间分析研究胸腺切除术后重症肌无力发病的预测因素。用时间回归分析法分析肌无力的演变。8名患者在胸腺切除术后平均33个月后出现晚期重症肌无力。年龄、性别、Masaoka分期和世界卫生组织的组织学没有发现显著相关性。

在单变量发病时间分析中，只有术前血清乙酰胆碱受体抗体滴度高（＞0.3 nmol/L）与胸腺切除术后重症肌无力显著相关（P=0.003）。正电子发射断层扫描通常在高滴度患者中进行，其中4名患者检测到代谢活性增加。在最后的这些病例中，通过再次胸骨切开术或电视胸腔镜进行了手术治疗，所有患者分别在12个月、24个月、32个月和48个月后病情缓解。目前还没有接受治疗的患者病情完全缓解。

在我们的研究中，术前高水平血清乙酰胆碱受体抗体的存在是唯一与胸腺切除术后重症肌无力发展显著相关的因素。异位胸腺组织残留胰岛的持续存在是肌无力发病的原因之一，手术切除是成功的。

Patients with thymoma and without clinical or electromyographical myasthenic signs may occasionally develop myasthenia several years after thymectomy. Hereby, we investigated the predictors and the evolution of this peculiar disease. We performed a retrospective analysis in 104 consecutive patients who underwent thymectomy between 1987 and 2013 for thymoma without clinical or electromyographic signs of myasthenia gravis. Predictors of post-thymectomy onset of myasthenia gravis were investigated with univariate time-to-disease analysis. Evolution of myasthenia was analyzed with time-to-regression analysis. Eight patients developed late myasthenia gravis after a median period of 33 months from thymectomy. No significant correlation was found for age, gender, Masaoka's stage, and World Health Organization histology. Only high preoperative serum acetylcholine-receptor antibodies titer (>0.3 nmol/L) was significantly associated with post-thymectomy myasthenia gravis at univariate time-to-disease (P = 0.003) analysis. Positron emission tomography was always performed in high-titer patients, and increased metabolic activity was detected in 4 of these patients. Surgical treatment through redo-sternotomy or video-assisted thoracoscopy was performed in these last cases with a remission in all patients after 12, 24, 32 and 48 months, respectively. No patient under medical treatment has yet developed a complete remission. In our study the presence of preoperative high-level serum acetylcholine receptor antibodies was the only factor significantly associated with the development of post-thymectomy myasthenia gravis. The persistence of residual islet of ectopic thymic tissue was one of the causes of the onset of myasthenia and its surgical removal was successful.

Keywords: acetylcholine receptor antibodies; myasthenia gravis; thymectomy; thymoma.

Mineo TC, Tamburrini A, Schillaci O, Ambrogi V. Onset and Evolution of Clinically Apparent Myasthenia Gravis After Resection of Non-myasthenic Thymomas. Semin Thorac Cardiovasc Surg. 2018 Summer;30(2):222-227. doi: 10.1053/j.semtcvs.2018.02.027. Epub 2018 Mar 6. PMID: 29522809.