Cherifi François, Dereeper Olivier, Forestier Alexandra, Joly Florence, Penel Nicolas
Caen Normandie University, Medical School, Caen, France.
Medical Oncology Department, Centre François Baclesse, Caen, France.
Chemotherapy. 2022;67(4):256-260. doi: 10.1159/000525154. Epub 2022 Jun 28.
Paraneoplastic neurological syndrome (PNS) is uncommon and not well known. PNS can reveal cancer, but its role in seminomas has not been described explicitly. We report the case of a 36-year-old man with unremarkable medical history and no comorbidities who was diagnosed with a retroperitoneal metastatic seminoma. The patient's general condition deteriorated, and he developed progressive neurological palsy without other clinical anomalies. Electromyography revealed demyelinating, non-lengthy neuropathy. Guillain-Barré syndrome was initially suspected. However, a positron emission tomography scan revealed a retroperitoneal mass, and blood markers revealed increased human chorionic gonadotropin. The patient was diagnosed with PNS, and a computed tomography-guided biopsy revealed a metastatic seminoma without a primary tumor. No circulating neural antibodies were detected. Human polyvalent immunoglobulin was simultaneously administered with chemotherapy. After three cycles of a cisplatin-etoposide-bleomycin, a complete biological and metabolic response rate was observed, and his neurological symptoms rapidly improved. Four years later, the patient responded completely, without any neurological complaints. Paraneoplastic demyelinating inflammatory neuropathy can lead to advanced seminoma diagnosis. Prompt management of seminomas with cisplatin-based regimens provides the best chance of cure for both advanced seminoma and paraneoplastic syndrome.
副肿瘤性神经系统综合征(PNS)并不常见,也鲜为人知。PNS可揭示癌症,但其在精原细胞瘤中的作用尚未得到明确描述。我们报告了一例36岁男性病例,其病史无异常且无合并症,被诊断为腹膜后转移性精原细胞瘤。患者的一般状况恶化,出现进行性神经麻痹且无其他临床异常。肌电图显示脱髓鞘性、非长度依赖性神经病变。最初怀疑为吉兰 - 巴雷综合征。然而,正电子发射断层扫描显示腹膜后肿块,血液标志物显示人绒毛膜促性腺激素升高。患者被诊断为PNS,计算机断层扫描引导下的活检显示为转移性精原细胞瘤,未发现原发性肿瘤。未检测到循环神经抗体。在化疗的同时给予人多价免疫球蛋白。在接受三个周期的顺铂 - 依托泊苷 - 博来霉素治疗后,观察到完全的生物学和代谢缓解率,其神经症状迅速改善。四年后,患者完全康复,无任何神经方面的主诉。副肿瘤性脱髓鞘性炎性神经病变可导致晚期精原细胞瘤的诊断。采用基于顺铂的方案对精原细胞瘤进行及时治疗,为晚期精原细胞瘤和副肿瘤综合征提供了最佳的治愈机会。