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资源有限环境下伴重症肌无力的晚期胸腺瘤的管理:一例报告

Management of Advanced Thymoma Presenting with Myasthenia Gravis in a Resource-limited Setting: A Case Report.

作者信息

Adnyana I Wayan Losen, Daniella Dian

机构信息

Division of Haematology and Oncology Medic, Department of Internal Medicine, Faculty of Medicine, University of Udayana / Prof. Dr. I.G.N.G. Ngoerah General Hospital, Denpasar, Bali, Indonesia.

Department of Internal Medicine, Faculty of Medicine, University of Udayana / Prof. Dr. I.G.N.G. Ngoerah General Hospital, Denpasar, Bali, Indonesia.

出版信息

Acta Med Philipp. 2025 Jan 15;59(1):110-115. doi: 10.47895/amp.vi0.8074. eCollection 2025.

DOI:10.47895/amp.vi0.8074
PMID:39897139
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11779666/
Abstract

Thymomas are rare tumours which generally account for only 0.2 - 1.5% of mediastinal tumours in adults. Around 40% of patients present with systemic symptoms such as motor weakness due to myasthenia gravis (MG), pure red cell aplasia, and hypogammaglobulinemia. Based on recent guidelines, management of advanced thymoma uses a multimodal approach, which is thymectomy followed by radiotherapy, but not all health care centers have radiotherapy facilities. A 52-year-old woman presented with nasal voice and had difficulty swallowing food. Patient was diagnosed with myasthenia gravis (MG). CT scan with contrast of the thorax showed a heterogenous solid mass in anterior mediastinum. Histopathological examination showed thymoma type B2. Thymectomy followed by seven cycles of platinum-based chemotherapy were done on the patient. Evaluation afterward showed complete remission of thymoma. The patient's motor weakness improved after the chemotherapy. Post-chemotherapy period was uneventful at six months on follow-up visit. The dosage of acetylcholinesterase inhibitor drug is reduced periodically due to improvement in motor weakness. The case emphasizes how to manage an advanced thymoma with MG with limited therapeutic options, and the importance of multidisciplinary management involving oncologists, surgeons, and neurologists.

摘要

胸腺瘤是罕见肿瘤,在成人纵隔肿瘤中通常仅占0.2 - 1.5%。约40%的患者会出现全身症状,如重症肌无力(MG)导致的运动无力、纯红细胞再生障碍和低丙种球蛋白血症。根据近期指南,晚期胸腺瘤的治疗采用多模式方法,即胸腺切除术后进行放疗,但并非所有医疗中心都有放疗设备。一名52岁女性出现鼻音且吞咽食物困难。患者被诊断为重症肌无力(MG)。胸部增强CT扫描显示前纵隔有一个不均匀实性肿块。组织病理学检查显示为B2型胸腺瘤。对该患者进行了胸腺切除术后给予七个周期的铂类化疗。术后评估显示胸腺瘤完全缓解。化疗后患者的运动无力症状有所改善。随访六个月,化疗后期间情况平稳。由于运动无力症状改善,乙酰胆碱酯酶抑制剂药物的剂量定期减少。该病例强调了在治疗选择有限的情况下如何管理合并MG的晚期胸腺瘤,以及肿瘤学家、外科医生和神经科医生参与多学科管理的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f04/11779666/316cbbf9ebc6/AMP-59-1-8074-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f04/11779666/b87d5f5bce15/AMP-59-1-8074-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f04/11779666/95feb924e74f/AMP-59-1-8074-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f04/11779666/b05496df56ea/AMP-59-1-8074-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f04/11779666/316cbbf9ebc6/AMP-59-1-8074-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f04/11779666/b87d5f5bce15/AMP-59-1-8074-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f04/11779666/95feb924e74f/AMP-59-1-8074-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f04/11779666/b05496df56ea/AMP-59-1-8074-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f04/11779666/316cbbf9ebc6/AMP-59-1-8074-g004.jpg

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本文引用的文献

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GOECP/SEOR radiotherapy guidelines for thymic epithelial tumours.GOECP/SEOR胸腺上皮肿瘤放射治疗指南。
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Thymoma Associated Myasthenia Gravis (TAMG): Differential Expression of Functional Pathways in Relation to MG Status in Different Thymoma Histotypes.胸腺瘤相关重症肌无力(TAMG):不同胸腺瘤组织类型中与 MG 状态相关的功能途径的差异表达。
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重症肌无力的胸腺切除术
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