胸腺瘤病理与重症肌无力预后

胸腺瘤病理与重症肌无力预后。Thymoma pathology and Myasthenia Gravis outcomes

引言：关于世界卫生组织(WHO)胸腺瘤亚型对胸腺瘤相关重症肌无力(TAMG)胸腺切除术后预后影响的证据有限。目的是确定胸腺瘤的病理亚型是否与TAMG患者胸腺切除术后重症肌无力(MG)的预后相关。

方法：我们对2018年1月至2019年12月期间在神经肌肉诊所就诊的连续TAMG患者进行了回顾性研究，胸腺切除术后至少随访1年。结果指标为MG损伤指数(MGII)、单一简单问题(SSQ)、美国重症肌无力基金会干预后状态(MGFA-PIS)和无应答者MG状态。

结果：95例患者，平均发病年龄48.1±12.1岁，女性54例(56.8%)。13例胸腺切除术后出现MG。最常见的胸腺瘤为WHO-B2型39例(41.1%)，大多数患者(40，42.1%)有MasaokaⅡ期胸腺瘤。胸腺瘤亚型或疾病的Masaoka分期与年龄、性别、MG表型、血清学、胸腺切除术后发病、发病至胸腺切除的时间间隔、MGII、SSQ、MGFA-PIS或无应答状态无关。血清学阳性与MGII较低(11.1±14.2 vs 23±12.9，p=0.050)、胸腺滤泡增生(TFH)和SSQ较高(89.3±11.7 vs 80.1±20.2，p-0.043)、无复发和SSQ较高(84.1±18 vs 72.5±20，p=0.037)之间存在相关性。

讨论：胸腺瘤的WHO病理亚型与MG预后无关。然而，乙酰胆碱抗体血清学阳性、TFH的存在和胸腺瘤的无复发可预测良好的预后。

关键词：重症肌无力;转归;分期;胸腺切除术;胸腺瘤亚型。

Introduction: There is limited evidence regarding the impact of World Health Organisation (WHO) subtype of thymoma on post-thymectomy outcome of thymoma-associated myasthenia gravis (TAMG). The objective was to determine if the pathological subtypes of thymoma were associated with post-thymectomy outcomes of myasthenia gravis (MG), in patients with TAMG.

Methods: We performed a retrospective study of consecutive patients with TAMG who attended the neuromuscular clinic between January 2018 and December 2019 with a minimum follow-up of 1 year after thymectomy. Outcome measures were MG impairment index (MGII), single-simple question (SSQ), Myasthenia Gravis Foundation of America post-intervention status (MGFA PIS) and non-responder MG status at last assessment.

Results: 95 patients were included; mean age at onset was 48.1 ± 12.1 years; 54(56.8%) were females. Thirteen patients developed MG post-thymectomy. The most common thymoma was WHO type B2 in 39(41.1%) Most patients (40, 42.1%) had Masaoka stage II thymoma. There was no association of thymoma subtypes or Masaoka stage of disease with age, gender, MG phenotype, serology, post-thymectomy onset, interval from onset to thymectomy, MGII, SSQ, MGFA PIS or non-responder status. Associations were found between positive serology and lower MGII (11.1 ± 14.2 vs 23 ± 12.9, p = 0.050), thymic follicular hyperplasia (TFH) and higher SSQ (89.3 ± 11.7 vs 80.1 ± 20.2, p-0.043), and lack of recurrence and higher SSQ (84.1 ± 18 vs 72.5 ± 20, p = 0.037).

Discussion: The WHO pathological subtype of thymoma did not correlate with MG outcomes. However, positive acetylcholine antibody serology, presence of TFH and non-recurrence of thymoma, predict a favourable outcome. This article is protected by copyright. All rights reserved.

Keywords: myasthenia gravis; outcomes; staging; thymectomy; thymoma subtype.