重症肌无力作为胰腺神经内分泌肿瘤的初始表现:一例报告。

Experimental and therapeutic medicine Pub Date : 2023-09-25 eCollection Date: 2023-11-01 DOI:10.3892/etm.2023.12222
Elena Stingu, Jerome-Maurice Dobrowolski, Paula Bombach, Dominik Nann, Stephan Singer, Marius Horger, Ulrich M Lauer, Lars Zender, Clemens Hinterleitner, Martina Hinterleitner
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引用次数: 0

摘要

重症肌无力(MG)是一种异质性自身免疫性疾病,其特征是突触后神经肌肉传递缺陷,抗体直接靶向乙酰胆碱受体(AChR)或神经肌肉接头的其他结构蛋白。大多数MG病例与胸腺病理有关,包括胸腺瘤、甲状腺炎、自身免疫性疾病或恶性血液系统肿瘤。本研究报告了一例罕见的AChR阳性和迟发性眼部MG病例,该病例迅速发展为全身性肌无力综合征,最初表现为胰腺神经内分泌肿瘤(pNEN)。pNEN完全手术切除后,肌无力综合征得到改善,抗AChR抗体滴度降低。据报道,MG是胸腺肿瘤中的一种副肿瘤综合征,在血液系统恶性肿瘤中不太常见。然而,目前,文献中只报道了少数MG作为实体瘤的初始表现,尤其是神经内分泌肿瘤。总之,在新诊断的MG患者中监测胸腺外实体恶性肿瘤可以促进相关肿瘤疾病的早期诊断。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Myasthenia gravis as initial presentation of a pancreatic neuroendocrine tumor: A case report.

Myasthenia gravis as initial presentation of a pancreatic neuroendocrine tumor: A case report.

Myasthenia gravis (MG) is a heterogeneous autoimmune disease, which is characterized by a postsynaptic neuromuscular transmission defect, with antibodies directly targeting the acetylcholine receptor (AChR) or other structural proteins of the neuromuscular junction. The majority of MG cases are associated with thymic pathologies, including thymoma, thyroiditis, autoimmune diseases or malignant hematologic neoplasia. The present study reported a rare case of AChR-positive and late-onset ocular MG, which rapidly progressed to a generalized myasthenic syndrome as an initial presentation of a pancreatic neuroendocrine neoplasia (pNEN). Following complete surgical resection of the pNEN, the myasthenic syndrome was improved and the anti-AChR antibody titers were reduced. It has been reported that MG is a paraneoplastic syndrome in thymic neoplasms and less common in hematologic malignancies. However, currently, only few cases of MG as initial presentation of a solid tumor, and more particular of a neuroendocrine neoplasm, have been reported in the literature. In conclusion, surveillance for extrathymic solid malignancies in newly diagnosed patients with MG could promote the early diagnosis of associated tumor diseases.

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