Paraneoplastic disorders in thymoma patients.
Paraneoplastic disorders in thymoma patients.
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胸腺瘤患者的副肿瘤疾病。
DOI：
10.1097/jto.0000000000000300
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发表时间：
2014-09
期刊：
影响因子：
--
通讯作者：
Lancaster E
中科院分区：
文献类型：
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作者：
Evoli A;Lancaster E
胸腺恶性肿瘤通常与副肿瘤性神经系统疾病（PND）有关，识别这些疾病对于治疗这些患者的医生来说非常重要。最常见的胸腺瘤相关PND是重症肌无力、获得性神经性肌强直（Isaacs综合征）、脑炎、Morvan综合征和肌炎。这些疾病的诊断是复杂的，但通常通过测试特异性自身抗体来辅助，包括针对重症肌无力的乙酰胆碱受体（AChR）和针对Caspr2（电压门控钾通道（VGKC）复合物的蛋白）的抗体，在患有获得性神经性肌强直、莫凡综合征或脑炎的患者中。表现出这些疾病的患者应在诊断时筛查胸腺瘤，这些PND的恶化可能与胸腺瘤复发有关。这些疾病可能导致严重残疾，但通常对免疫疗法有反应，并且经常通过胸腺瘤治疗得到改善。需要一个专家团队的密切合作，以适当地照顾这些病人。
Thymic malignancy is often associated with paraneoplastic neurological diseases (PNDs) and recognition of these disorders is important for physicians who treat these patients. The most common thymoma-associated PNDs are myasthenia gravis, acquired neuromyotonia (Isaacs' syndrome), encephalitis, Morvan's syndrome, and myositis. Diagnosis of these disorders is complex but often aided by testing for specific autoantibodies, including those to the acetylcholine receptor (AChR) for myasthenia gravis and to Caspr2, protein of the voltage-gated potassium channel (VGKC) complex, in patients with acquired neuromyotonia, Morvan's syndrome, or encephalitis. Patients who manifest these disorders should be screened for thymoma at diagnosis, and worsening of these PNDs may be associated with recurrent thymoma. These disorders can cause profound disability but usually respond to immunotherapy, and often improve with thymoma treatment. Close cooperation among a team of specialists is required to properly care for these patients.