Regionally Metastatic Merkel Cell Carcinoma Associated with Paraneoplastic Anti-N-methyl-D-aspartate Receptor Encephalitis.
Regionally Metastatic Merkel Cell Carcinoma Associated with Paraneoplastic Anti-N-methyl-D-aspartate Receptor Encephalitis.
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DOI：
10.1155/2020/1257587
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发表时间：
2020
影响因子：
0.9
通讯作者：
Miller DM
中科院分区：
文献类型：
--
作者：
Shalhout SZ;Emerick KS;Sadow PM;Linnoila JJ;Miller DM
默克尔细胞癌（MCC）是一种罕见的侵袭性皮肤神经内分泌癌，具有很高的复发和转移风险。MCC通常与高龄、皮肤白皙、日晒、免疫抑制以及在大多数情况下与默克尔细胞多瘤病毒有关。神经内分泌恶性肿瘤与多种副肿瘤神经综合征（PNS）相关，其特征是对恶性肿瘤相关神经抗原表达的自身免疫反应。我们的文献回顾强调了之前报道的mcc相关PNS伴有电压门控钙通道（VGCC）和抗hu（或ANNA-1）自身抗体的病例。我们报告一个59岁的男性，局部转移性默克尔细胞癌合并抗n -甲基- d -天冬氨酸受体（NMDAR）脑炎的副肿瘤表现。他的原发性下颈部皮下MCC和转移最初用手术治疗。其他复发性淋巴结转移成功地治疗了明确的调强放疗。利妥昔单抗治疗改善了PNS。虽然罕见，但本病例强调，在癫痫发作和显著精神症状伴随MCC诊断的情况下，评估自身免疫性副肿瘤脑炎是有必要的。在晚期MCC的免疫检查点抑制剂（ICI）时代，预先存在的PNS的意识和检测是至关重要的，ICI治疗有可能加剧预先存在的自身免疫性PNS，导致恶化甚至致命的神经免疫相关不良事件（nirAEs）。
Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous neuroendocrine cancer with a high risk of recurrence and metastasis. MCC is generally associated with advanced age, fair skin, sun exposure, immunosuppression, and in the majority of cases, the Merkel cell polyomavirus. Neuroendocrine malignancies are associated with a variety of paraneoplastic neurological syndromes (PNS), characterized as autoimmune responses to malignancy-associated expression of neural antigens. Our literature review underscores previous case reports of MCC-associated PNS with voltage-gated calcium channel (VGCC) and anti-Hu (or ANNA-1) autoantibodies. We present the case of a 59-year-old male with regionally metastatic Merkel cell carcinoma complicated by the paraneoplastic manifestation of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. His primary lower neck subcutaneous MCC and metastasis were initially treated with surgery. Additional recurrent lymph node metastases were successfully treated with definitive intensity-modulated radiation therapy. His PNS improved with rituximab therapy. Although rare, this case highlights that in the setting of seizures and prominent psychiatric symptoms accompanying an MCC diagnosis, evaluation for autoimmune paraneoplastic encephalitis is warranted. Awareness and detection of preexisting PNS are crucial in the era of immune checkpoint inhibitors (ICI) for advanced MCC, where treatment with ICI has the potential to exacerbate preexisting autoimmune PNS and lead to worsened or even lethal neurologic immune-related adverse events (nirAEs).