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胸腺瘤合并重症肌无力:病例报告。

Ectopic cervical thymoma in myasthenia gravis: a case report.

机构信息

Department of Neurology, The University of Texas Health Sciences Center at San Antonio, San Antonio, TX, USA.

Department of Neurology, University of New Mexico Hospital, Albuquerque, NM, USA.

出版信息

BMC Neurol. 2024 May 23;24(1):173. doi: 10.1186/s12883-024-03656-6.

Abstract

BACKGROUND

Ectopic cervical thymoma (ECT) is an extremely rare tumor, especially in association with myasthenia gravis (MG).

CASE PRESENTATION

We report a case of myasthenia gravis with an ectopic thymoma in the neck, whose myasthenic symptoms significantly improved after complete removal of the mass. A 55-year-old woman with generalized myasthenia gravis (MG) experienced worsening neuromuscular weakness after abruptly discontinuing pyridostigmine. Testing revealed acetylcholine receptor-antibody (AChR-Ab) positivity and a cervical mass initially thought to be thyroid or parathyroid was identified as a thymoma, type A. Post-surgery and radiation therapy, her myasthenic symptoms improved significantly with less prednisone and pyridostigmine requirements over time and no need for additional immunotherapies.

CONCLUSIONS

Diagnosing ECTs is challenging due to rarity, atypical locations, and inconclusive fine needle aspiration cytology (FNAC) results, often misinterpreted as thyroid or parathyroid lesions. As proper management of patients with MG, including thymectomy, offers favorable clinical outcomes such as significant improvement in myasthenic complaints and reduced immunosuppressive medication requirements, clinicians should be vigilant of the ectopic locations of thymomas to ensure timely diagnosis and intervention.

摘要

背景

异位性颈胸腺肿瘤(ECT)是一种极其罕见的肿瘤,特别是与重症肌无力(MG)相关联时。

病例介绍

我们报告了一例伴有异位胸腺肿瘤的重症肌无力病例,该患者在完全切除肿块后肌无力症状明显改善。一名 55 岁女性患有全身性重症肌无力(MG),在突然停用吡啶斯的明后出现神经肌肉无力恶化。检测显示乙酰胆碱受体抗体(AChR-Ab)阳性,最初被认为是甲状腺或甲状旁腺的颈部肿块被确定为 A 型胸腺瘤。手术后和放射治疗后,随着时间的推移,她的肌无力症状显著改善,泼尼松和吡啶斯的明的需求减少,并且不需要额外的免疫治疗。

结论

由于罕见性、非典型位置和不明确的细针穿刺细胞学(FNAC)结果,ECT 的诊断具有挑战性,往往被误诊为甲状腺或甲状旁腺病变。由于对 MG 患者进行适当的治疗,包括胸腺切除术,可以提供有利的临床结果,例如肌无力症状显著改善和减少免疫抑制药物的需求,因此临床医生应警惕胸腺肿瘤的异位位置,以确保及时诊断和干预。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24f6/11112909/141917d4e81a/12883_2024_3656_Fig1_HTML.jpg

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