Oyanagi Masahiko, Kakiuchi Akito, Yamamoto Keisuke, Kurose Makoto, Takano Kenichi
Otolaryngology-Head and Neck Surgery, Sapporo Medical University School of Medicine, Sapporo, JPN.
Cureus. 2025 Aug 16;17(8):e90210. doi: 10.7759/cureus.90210. eCollection 2025 Aug.
Paraneoplastic neurological syndromes (PNS) are rare, immune-mediated disorders associated with cancer, often presenting with various neurological symptoms, such as encephalomyelitis, limbic encephalitis, and rapidly progressive cerebellar syndrome. A 66-year-old woman presented to our department with rapidly increasing swelling in her right parotid gland. Fine needle aspiration cytology confirmed parotid cancer, and a right parotidectomy and dermectomy were planned. Preoperatively, she experienced symptoms of nausea and vertigo and was later diagnosed with PNS based on the presence of Zic4 antibodies in the cerebrospinal fluid. Following methylprednisolone pulse therapy and tumor resection, the patient's symptoms resolved completely. Histopathological examination of the surgically excised tissues confirmed a diagnosis of salivary duct carcinoma. Immunohistochemistry revealed that the Zic4 antibody stained the nucleoli of tumor cells. This report presents the first documented case of PNS linked to parotid cancer expressing the Zic4 antibody, along with a review of 21 previous cases of PNS associated with head and neck cancers.
副肿瘤性神经系统综合征（PNS）是罕见的、与癌症相关的免疫介导性疾病，常表现为各种神经系统症状，如脑脊髓炎、边缘叶脑炎和快速进展性小脑综合征。一名66岁女性因右侧腮腺迅速肿大前来我院就诊。细针穿刺细胞学检查确诊为腮腺癌，计划行右侧腮腺切除术和皮肤切除术。术前，她出现恶心和眩晕症状，后来根据脑脊液中存在Zic4抗体被诊断为PNS。经过甲泼尼龙冲击治疗和肿瘤切除后，患者症状完全缓解。手术切除组织的组织病理学检查确诊为涎腺导管癌。免疫组织化学显示，Zic4抗体对肿瘤细胞核仁进行了染色。本报告介绍了首例记录在案的与表达Zic4抗体的腮腺癌相关的PNS病例，并对之前21例与头颈癌相关的PNS病例进行了综述。
