Department of Histopathology, King Edward Memorial Hospital, Perth, Western Australia.
Int J Gynecol Pathol. 2012 Sep;31(5):429-37. doi: 10.1097/PGP.0b013e31824a1de2.
Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a recently described severe neurological disorder predominantly affecting young women, which presents with psychosis, memory deficits, seizures, and encephalopathy, often requiring prolonged hospitalization. The condition is frequently associated with an underlying neoplasm, most often an ovarian teratoma, and in such cases appears to be a para-neoplastic, immune-mediated encephalopathy. The histologic features of the teratomas associated with anti-NMDAR encephalitis have seldom been described in detail. Therefore, in this report, we have compared ovarian teratomas (4 mature and 1 immature) from 5 patients with anti-NMDAR encephalitis with 22 sporadic control teratomas (14 mature and 8 immature) that included neuroglial elements. The encephalitis-associated tumors ranged from 0.7 to 9.5 cm diameter, and 1 case was bilateral; the second teratoma was discovered 13 mo after the first when symptoms recurred. In comparison with control teratomas, the anti-NMDAR-associated tumors showed a more marked intratumoral lymphoid infiltrate that colocalized to the mature neuroglial elements. Reactive germinal centers (3 cases) and diffuse lymphoplasmacytic infiltrates within the neuroglial matrix (4 cases), and degenerative neuronal changes (2 cases), were seen only in the anti-NMDAR-positive cases. Pathologists encountering ovarian teratomas with these distinctive reactive lymphoid elements should consider the possibility of anti-NMDAR encephalitis, particularly because the neurological symptoms may develop after tumor resection. Careful histopathologic examination may be required to identify small, radiologically occult teratomas, and to demonstrate the presence of subtle neoplastic neuroglial components in teratomas associated with anti-NMDAR encephalitis.
抗 N-甲基-D-天冬氨酸受体（NMDAR）脑炎是一种新近描述的严重神经系统疾病，主要影响年轻女性，表现为精神病、记忆缺陷、癫痫发作和脑病，常需要长期住院治疗。这种情况常与潜在的肿瘤有关，最常见的是卵巢畸胎瘤，在这种情况下，它似乎是一种副瘤性、免疫介导的脑病。与抗 NMDAR 脑炎相关的畸胎瘤的组织学特征很少有详细描述。因此，在本报告中，我们比较了 5 例抗 NMDAR 脑炎患者的卵巢畸胎瘤（4 例成熟和 1 例不成熟）与 22 例散发性对照畸胎瘤（14 例成熟和 8 例不成熟），包括神经胶质成分。脑炎相关肿瘤的直径为 0.7 至 9.5 厘米，1 例为双侧；第 2 个畸胎瘤是在第 1 个症状复发 13 个月后发现的。与对照畸胎瘤相比，抗 NMDAR 相关肿瘤显示出更明显的肿瘤内淋巴浸润，与成熟神经胶质成分共定位。在抗 NMDAR 阳性病例中仅观察到 3 例反应性生发中心（3 例）和神经胶质基质内弥漫性淋巴浆细胞浸润（4 例），以及 2 例退行性神经元变化。遇到具有这些独特反应性淋巴样成分的卵巢畸胎瘤的病理学家应考虑抗 NMDAR 脑炎的可能性，特别是因为神经系统症状可能在肿瘤切除后发生。可能需要仔细的组织病理学检查来识别小的、影像学隐匿的畸胎瘤，并在抗 NMDAR 脑炎相关的畸胎瘤中证明存在微妙的肿瘤性神经胶质成分。
