Neurolymphomatosis of cranial nerves as the responsible lesions for the vocal cord paralysis and facial nerve palsy in a patient with diffuse large B-cell lymphoma
Neurolymphomatosis of cranial nerves as the responsible lesions for the vocal cord paralysis and facial nerve palsy in a patient with diffuse large B-cell lymphoma
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颅神经神经淋巴瘤病是弥漫性大 B 细胞淋巴瘤患者声带麻痹和面神经麻痹的主要病变
DOI：
10.1007/s00277-022-05038-9
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发表时间：
2022
影响因子：
3.5
通讯作者：
Kuroda Junya
中科院分区：
文献类型：
--
作者：
Inoue Yu;Hirakawa Koichi;Hayata Hiroki;Nishiyama Daichi;Fujino Takahiro;Tsukamoto Taku;Mizutani Shinsuke;Shimura Yuji;Kuroda Junya
在此我们报告一个弥漫性大B细胞淋巴瘤的病例，包括分别由右侧迷走神经和面神经的神经淋巴瘤病所引起的右侧声带麻痹和面神经麻痹。作为初步诊断，一名60岁的男性患者被确定为患有DLBCL，根据安阿伯分期系统为IV期。患者接受了6个周期的利妥昔单抗（R）-CHOP治疗，18 F-氟脱氧葡萄糖正电子发射断层扫描结合计算机断层扫描（18 F-FDG-PET/CT）显示患者实现了完全代谢反应。然而，在完成R-CHOP后三个月，患者出现右侧声带麻痹和随后的右侧面瘫。脑钆对比磁共振成像（MRI）扫描可见右侧面神经和右侧迷走神经增强，无任何可能导致右侧喉返神经或面神经肿块病变的异常（图1）。当在脑脊液（CSF）中检测到淋巴瘤细胞时，全身CT扫描显示下腹部皮下病变出现复发性肿瘤。右侧迷走神经和右侧面神经出现NL，以及这些症状，表明DLBCL复发，尽管我们跳过了组织学评估以避免侵入性操作。患者接受R-MA治疗，包括第1天利妥昔单抗375 mg/m2，第2天甲氨蝶呤（MTX）3.5 g/m2，第3天和第4天阿糖胞苷（CA）2 g/m2，每日两次，同时鞘内注射MTX 15 mg/体、CA 40 mg/体和泼尼松龙10 mg/体。声音嘶哑和面瘫暂时改善，但疾病很快复发，并对许多遗传毒性药物和polatuzumab vedotin耐药。由于对一系列细胞毒性化疗缺乏反应和全身快速恶化，患者不适合接受自体或同种异体干细胞移植的高剂量化疗。患者最终在疾病复发后6个月死亡。由于NL是DLBCL患者中的罕见事件，NL的诊断并不总是简单的，并且在DLBCL的日常实践中经常被延迟[1-6]。此外，NL偶尔先于全身性淋巴瘤累及出现，约占NL病例的25 - 50%。这再次强调了将NL视为病因不明的神经系统症状的原因的重要性，即使在没有已知淋巴瘤病史的患者中也是如此[2，4，7，8]。由于神经活检具有运动和感觉缺陷的潜在风险，并且由于淋巴瘤细胞的斑片状神经浸润而在NL诊断中具有挑战性[2，6，8]，因此NL诊断的关键替代策略是放射学评价。即使在没有异常MRI结果的患者中，18F-FDG-PET/CT也是诊断NL的敏感诊断方法[2，4]。然而，由于18F-FDG在大脑中的生理摄取，因此颅神经NL的诊断具有挑战性。为了平衡它们的优点和缺点，建议使用两种成像方式诊断NL [8]。不幸的是，我们的病例没有进行18F-FDG-PET/CT。
Here we report a case of diffuse large B-cell lymphoma (DLBCL) that included right vocal cord paralysis and facial palsy brought on by neurolymphomatosis (NL) of the right vagus nerve and facial nerve, respectively. As the initial diagnosis, a 60-year-old male patient was identified as having DLBCL, stage IV according to the Ann Arbor staging system. The patient was subjected to six cycles of rituximab (R)-CHOP therapy, and 18F-fluorodeoxyglucose positron emission tomography combined with computed tomography (18F-FDG-PET/CT) revealed that the patient achieved complete metabolic response. Three months after finishing R-CHOP, however, the patient suffered from right vocal cord paralysis and the subsequent right facial palsy. The right facial nerve and right vagus nerve enhancement were seen on a brain gadolinium-contrast magnetic resonance imaging (MRI) scan without any abnormalities that could have caused mass lesions in the right recurrent laryngeal nerve or facial nerve (Fig. 1). When lymphoma cells were detected in the cerebrospinal fluid (CSF), a systemic CT scan revealed the emergence of a relapsed tumor in the lower abdominal subcutaneous lesion. The emergence of NL in the right vagus nerve and right facial nerve, together with these symptoms, pointed to a relapse of DLBCL, though we skipped the histologic evaluation to avoid the invasive procedure. The patient received R-MA therapy, comprising rituximab 375 mg/m2 on day 1, methotrexate (MTX) 3.5 g/m2 on day 2, and cytarabine (CA) 2 g/m2 twice a day on days 3 and 4, with concurrent intrathecal injection of MTX 15 mg/body, CA 40 mg/body, and prednisolone 10 mg/body. Hoarseness and facial palsy temporarily improved, but the disease quickly returned and was resistant to many genotoxic agents and polatuzumab vedotin. The patient was ineligible for high dose chemotherapy with autologous or allogeneic stem cell transplantation due to the lack of response to series of cytotoxic chemotherapy and rapid general deterioration. The patient eventually died 6 months after the disease’s relapse. Because NL is a rare event in patients with DLBCL, the diagnosis of NL is not always simple and has frequently been delayed in the daily practice of DLBCL [1–6]. Additionally, NL occasionally precedes the emergence of systemic lymphoma involvement, which accounts for roughly 25 to 50% of cases with NL. The significance of considering NL as the cause of neurologic symptoms with unknown etiology, even in patients without a known history of lymphoma, is once again highlighted by this [2, 4, 7, 8]. Because the nerve biopsy has the potential risk of motor and sensory deficits and is challenging due to patchy nerve infiltration of lymphoma cells in the diagnosis of NL [2, 6, 8], the critical alternative strategy for the diagnosis of NL is the radiologic evaluation. Even in patients without abnormal MRI findings, 18F-FDG-PET/CT is a sensitive diagnostic modality for the diagnosis of NL [2, 4]. However, because of the physiological uptake of 18F-FDG in the brain, the diagnosis of NL in the cranial nerve is challenging. To balance out their advantages and disadvantages, it is advised to use both imaging modalities for the diagnosis of NL [8]. Sadly, 18F-FDG-PET/CT was not done in our case.
周围神经系统神经淋巴瘤病：病例报告和文献综述。
影响因子：
2.1
作者：
Panagiotis A. Sideras;J. Matthews;S M Nazmus Sakib;Franka Ofikwu;V. Spektor
通讯作者：
V. Spektor