Spontaneous resolution of metastatic thymoma with prednisone in a patient with juvenile myasthenia gravis: a case report

Shanghai chest Pub Date : 2021-01-01 DOI:10.21037/shc-22-15
N. Ramesh, J. Velotta
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Abstract

Background: Juvenile myasthenia gravis is a rare autoimmune condition affecting children. Similar to myasthenia in adults, juvenile myasthenia gravis is associated with thymomas and many of these patients undergo thymectomy. However, there is no clear consensus on which patients could benefit from surgical intervention and how to manage refractory disease. Case Description: In this report, we describe a young boy who was diagnosed with myasthenia gravis and found to have stage IVa type B2 metastatic thymoma. Despite numerous pharmaceutical interventions, including neoadjuvant chemotherapy, his disease continued to progress. He ultimately underwent an extended radical parietal and visceral pleurectomy with decortication, total thymectomy, partial pericardial resection, and diaphragm resection and reconstruction. However, he had recurrence of his thymoma and was started on prednisone for palliative intent. Remarkably, within a few months he was noted to have interval decrease in his thoracic tumor burden and near resolution of his metastatic disease. Conclusions: Although spontaneous remission has been described in pediatric myasthenia, this report sheds light on possible management strategies post-thymectomy. This case additionally underscores the varied outcomes in patients treated for juvenile myasthenia gravis. Ultimately, this case, when added to both existing and future studies, will inform the development of guidelines on pediatric myasthenia gravis treatment. been well characterized. In this report, we describe a rare case of severe JMG and metastatic thymoma that responded to steroid therapy, highlighting the potential role of corticosteroids for JMG and thymoma refractory to thymectomy. We present the following case in accordance with the CARE reporting checklist (available at https://shc.
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原发性重症肌无力患者转移性胸腺瘤经强的松治疗后自发性消退1例
背景:青少年重症肌无力是一种罕见的影响儿童的自身免疫性疾病。与成人重症肌无力相似,青少年重症肌无力与胸腺瘤有关,许多患者接受胸腺切除术。然而,对于哪些患者可以从手术干预中获益以及如何处理难治性疾病,目前还没有明确的共识。病例描述:在这个报告中,我们描述了一个年轻的男孩,他被诊断为重症肌无力,发现有IVa期B2型转移性胸腺瘤。尽管许多药物干预,包括新辅助化疗,他的疾病继续发展。他最终接受了扩展根治性胸膜壁和内脏切除术,包括去皮、全胸腺切除术、部分心包切除术和膈切除术和重建。然而,他的胸腺瘤复发,并开始使用强的松缓解意图。值得注意的是,在几个月内,他的胸部肿瘤负荷间隔减少,转移性疾病接近消退。结论:虽然在小儿重症肌无力中有自发性缓解的描述,但本报告揭示了胸腺切除术后可能的管理策略。该病例进一步强调了青少年重症肌无力患者治疗的不同结果。最终,当该病例加入到现有和未来的研究中时,将为儿童重症肌无力治疗指南的制定提供信息。被很好地描述了。在这篇报道中,我们描述了一个罕见的严重JMG和转移性胸腺瘤对类固醇治疗有反应的病例,强调了皮质类固醇对JMG和胸腺切除术难治的胸腺瘤的潜在作用。我们根据CARE报告清单(可在https://shc上找到)提出以下病例。
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