Medical College of Georgia, Augusta, GA.
Department of Pathology, Medical College of Georgia, Augusta, GA.
Yale J Biol Med. 2020 Sep 30;93(4):495-500. eCollection 2020 Sep.
Testicular tumors account for 1-2% of all tumors in men, with 95% of these being germ cell tumors. Paraneoplastic limbic encephalitis is a rare sequela of testicular tumors associated with anti-Ma2 and KLH11 antibodies. The most effective treatment for paraneoplastic limbic encephalitis is treatment of the primary malignancy. We report a 41-year-old male that presented to the emergency department with episodic alteration of consciousness and memory disturbances. Negative neurologic evaluation and imaging led to concern for a paraneoplastic process from a distant malignancy. CT imaging revealed an enlarged, necrotic para-aortic lymph node and subsequent ultrasound demonstrated a right-sided testicular mass. Right radical orchiectomy was performed. Microscopically, the mass consisted of mixed respiratory epithelium, gastrointestinal glands, and squamous epithelium with keratinization consistent with a post-pubertal testicular teratoma with associated germ cell neoplasia. Resection of the para-aortic mass revealed large anaplastic cells with epithelioid features, nuclear pleomorphism and frequent mitoses. Immunostaining was positive for Pan-Keratin and OCT4, consistent with poorly differentiated embryonal carcinoma. Resection of the primary and metastatic disease, as well as treatment with corticosteroids, resulted in resolution of the encephalitis. This presentation of severe neurological disturbances in the setting of a metastatic mixed non-seminomatous germ cell tumor represents a rare presentation of paraneoplastic limbic encephalitis.
睾丸肿瘤占男性所有肿瘤的 1-2%，其中 95%为生殖细胞肿瘤。副肿瘤性边缘叶脑炎是一种罕见的睾丸肿瘤相关后遗症，与抗 Ma2 和 KLH11 抗体有关。副肿瘤性边缘叶脑炎的最有效治疗方法是治疗原发性恶性肿瘤。我们报告了一名 41 岁男性，因意识障碍和记忆障碍反复发作而到急诊科就诊。阴性的神经系统评估和影像学检查提示远处恶性肿瘤的副肿瘤过程。CT 成像显示增大的坏死性主动脉旁淋巴结，随后的超声显示右侧睾丸肿块。进行了右侧根治性睾丸切除术。显微镜下，肿块由混合呼吸上皮、胃肠道腺体和角化的鳞状上皮组成，符合青春期后睾丸畸胎瘤伴生殖细胞肿瘤。主动脉旁肿块切除术显示大的间变性细胞，具有上皮样特征、核多形性和频繁有丝分裂。免疫组化染色为 Pan-Keratin 和 OCT4 阳性，符合低分化胚胎癌。切除原发性和转移性疾病，并使用皮质类固醇治疗，导致脑炎消退。这种转移性混合非精原细胞瘤生殖细胞肿瘤的严重神经功能障碍表现代表了副肿瘤性边缘叶脑炎的一种罕见表现。
