Abstract
Ovarian teratomas are by far the most common ovarian germ cell tumor. Most teratomas are benign unless a somatic transformation occurs. The designation of teratoma refers to a neoplasm that differentiates toward somatic-type cell populations. Recent research shows a striking association between ovarian teratomas and anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, a rare and understudied paraneoplastic neurological syndrome (PNS). Among teratomas, mature teratomas are thought to have a greater relevance with those neurological impairments. PNS is described as a neurologic deficit triggered by an underlying remote tumor, whereas anti-NMDAR encephalitis is characterized by a complex neuropsychiatric syndrome and the presence of autoantibodies in cerebral spinal fluid against the GluN1 subunit of the NMDAR. This review aims to summarize recent reports on the association between anti-NMDAR encephalitis and ovarian teratoma. In particular, the molecular pathway of pathogenesis and the updated mechanism and disease models would be discussed. We hope to provide an in-depth review of this issue and, therefore, to better understand its epidemiology, diagnostic approach, and treatment strategies.
Highlights
Ovarian TeratomaOvarian teratomas are the most common ovarian germ cell tumors (GCTs), and among all teratomas, the most frequently occurring ovarian GCTs are benign, cystic mature teratomas (MTs) [1,2]
Since first reported 14 years ago, anti-NMDAR encephalitis has gained a great of attention and become recognized as a rare but treatable autoimmune disorder, with about
This article reviewed studies from 2007 to 2020 and concluded some information regarding clinicopathological characteristics, such as age distribution, histological property, symptoms at presentation, and relapse rate. Though this entity’s incidence varies in different studies, we have concluded that the overall incidence of ovarian teratoma among anti-NMDAR encephalitis patients is 37.4%
Summary
Ovarian teratomas are the most common ovarian germ cell tumors (GCTs), and among all teratomas, the most frequently occurring ovarian GCTs are benign, cystic mature teratomas (MTs) [1,2]. The last classification of teratomas is monodermal highly specialized teratomas, closely associated with MTs that consist of a predominant mature histologic cell type [2,15] This rare and remarkable subset of teratomas may show a broad range of morphologies, such as struma ovarii, carcinoid neoplasms, sebaceous gland tumors, and neurogenic cysts [2,4]. PNS is a heterogeneous group of disorders that may occur with any malignancy, it is more commonly associated with small cell carcinoma, ovarian cancer, breast cancer, neuroendocrine tumors, thymoma, and lymphoma [18]. Anti-NMDAR encephalitis is associated with various tumors, such as MTs, mediastinal teratoma, small cell lung cancer (SCLC), and ovarian cystadenofibroma, while MTs are the most associated [8,28]. Abnormal (psychiatric) behavior or cognitive dysfunction; Speech dysfunction (pressured speech, verbal reduction, or mutism); Seizures; Movement disorder, dyskinesias, rigidity, or abnormal postures; Decreased consciousness; Autonomic dysfunction or central hypoventilation
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