Paraneoplastic Syndromes Associated with Ovarian Teratomas - Systematic Review
DOI:
https://doi.org/10.12775/QS.2026.54.70398Keywords
ovarian teratoma, paraneoplastic syndromes, anti-NMDAR encephalitis, autoimmune disorders, germ cell tumors, mature teratoma, immature teratomaAbstract
Ovarian teratomas are germ cell tumors characterized by their ability to differentiate into tissues derived from all three embryonic germ layers. Although most teratomas are benign, particularly mature cystic teratomas, they may be associated with a wide spectrum of paraneoplastic syndromes. These syndromes are not caused by the direct presence of the tumor but rather by immune-mediated or hormonal mechanisms triggered by the neoplasm. The most well-recognized association is anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, although other neurological, hematological, and systemic manifestations have also been described.
This systematic review aims to analyze the types of paraneoplastic syndromes associated with ovarian teratomas, their pathophysiology, clinical presentation, and diagnostic significance. Particular attention is given to neurological autoimmune syndromes, which often precede tumor diagnosis and may serve as an early clinical indicator of an underlying neoplasm. Understanding these associations is essential for timely diagnosis and appropriate management, which frequently involves a combination of tumor resection and immunotherapy.
References
1. Cong L, Wang S, Yeung SY, Lee JHS, Chung JPW, Chan DYL. Mature Cystic Teratoma: An Integrated Review. Int J Mol Sci. 24 marca 2023;24(7):6141. doi:10.3390/ijms24076141 PubMed PMID: 37047114; PubMed Central PMCID: PMC10093990.
2. Ayhan A, Bukulmez O, Genc C, Karamursel BS, Ayhan A. Mature cystic teratomas of the ovary: case series from one institution over 34 years. Eur J Obstet Gynecol Reprod Biol. lutego 2000;88(2):153–7. doi:10.1016/S0301-2115(99)00141-4
3. Landolfo C, Froyman W, Testa AC, Fischerova D, Franchi D, Yazbek J, et al. Imaging in gynecological disease (29): clinical and ultrasound features of primary ovarian immature teratoma. Ultrasound Obstet Gynecol Off J Int Soc Ultrasound Obstet Gynecol. stycznia 2026;67(1):89–99. doi:10.1002/uog.70111 PubMed PMID: 41118662; PubMed Central PMCID: PMC12757824.
4. Vorasart P, Aroonroch R, Rermluk N, Vallibhakara O, Sriphrapradang C. Papillary Thyroid Carcinoma Arising Within a Mature Ovarian Cystic Teratoma: A Case Report. Case Rep Endocrinol. 2025;2025:7914933. doi:10.1155/crie/7914933 PubMed PMID: 40746588; PubMed Central PMCID: PMC12313371.
5. Chao A, Lai CH, Chao AS, Lin CY, Wang YC, Huang HJ, et al. Molecular characterization of ovarian squamous cell carcinoma originating from mature teratoma. J Mol Med. stycznia 2025;103(1):101–11. doi:10.1007/s00109-024-02505-w
6. Aziz A, Ahuja S, Ahluwalia C. Bilateral ovarian mature cystic teratoma with unilateral malignant transformation to adenocarcinoma: A case report. J Cancer Res Ther. stycznia 2025;21(1):266–9. doi:10.4103/jcrt.jcrt_448_23
7. Lin J, Wang M, Wang J, Li J. Ovarian Teratoma-Related Paraneoplastic Neurological Syndromes. Front Oncol. 2022;12:892539. doi:10.3389/fonc.2022.892539 PubMed PMID: 35651803; PubMed Central PMCID: PMC9149209.
8. Dalmau J. NMDA receptor encephalitis and other antibody-mediated disorders of the synapse: The 2016 Cotzias Lecture. Neurology. 6 grudnia 2016;87(23):2471–82. doi:10.1212/WNL.0000000000003414
9. Dalmau J, Armangué T, Planagumà J, Radosevic M, Mannara F, Leypoldt F, et al. An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. Lancet Neurol. listopada 2019;18(11):1045–57. doi:10.1016/S1474-4422(19)30244-3 PubMed PMID: 31326280.
10. Althobaiti S, Assinnari A, Alharbi M, Kurdi R. Case Report: Autoimmune hemolytic anemia associated with ovarian teratoma in a 13-year-old: a rare paraneoplastic presentation. Front Pediatr. 27 października 2025;13:1700443. doi:10.3389/fped.2025.1700443
11. Tabata E, Masuda M, Eriguchi M, Yokoyama M, Takahashi Y, Tanaka K, et al. Immunopathological significance of ovarian teratoma in patients with anti-N-methyl-d-aspartate receptor encephalitis. Eur Neurol. 2014;71(1–2):42–8. doi:10.1159/000353982 PubMed PMID: 24296881.
12. Kim I, Lee JY, Kwon JH, Jung JY, Song HH, Park YL, et al. A Case of Autoimmune Hemolytic Anemia Associated with an Ovarian Teratoma. J Korean Med Sci. 2006;21(2):365. doi:10.3346/jkms.2006.21.2.365
13. Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JCG, Desestret V, Dubey D, et al. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. Neurol Neuroimmunol Neuroinflammation. lipca 2021;8(4):e1014. doi:10.1212/NXI.0000000000001014
14. Kunchok A, Zekeridou A, McKeon A. Autoimmune glial fibrillary acidic protein astrocytopathy. Curr Opin Neurol. czerwca 2019;32(3):452–8. doi:10.1097/WCO.0000000000000676
15. Fang B, McKeon A, Hinson SR, Kryzer TJ, Pittock SJ, Aksamit AJ, et al. Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Novel Meningoencephalomyelitis. JAMA Neurol. 1 listopada 2016;73(11):1297. doi:10.1001/jamaneurol.2016.2549
16. Talukdar S, Kumar S, Bhatla N, Mathur S, Thulkar S, Kumar L. Neo-adjuvant chemotherapy in the treatment of advanced malignant germ cell tumors of ovary. Gynecol Oncol. stycznia 2014;132(1):28–32. doi:10.1016/j.ygyno.2013.10.009 PubMed PMID: 24145115.
17. Cao M, Deng Y, Deng Y, Wu J, Yang C, Wang Z, et al. Characterization of immature ovarian teratomas through single-cell transcriptome. Front Immunol. 3 marca 2023;14:1131814. doi:10.3389/fimmu.2023.1131814
18. Ren X, Guo Z, Bai J. Struma ovarii with contralateral ovarian teratoma: A case report. Front Surg. 19 sierpnia 2022;9:907326. doi:10.3389/fsurg.2022.907326
19. Sepúlveda M, Sola-Valls N, Escudero D, Rojc B, Barón M, Hernández-Echebarría L, et al. Clinical profile of patients with paraneoplastic neuromyelitis optica spectrum disorder and aquaporin-4 antibodies. Mult Scler J. listopada 2018;24(13):1753–9. doi:10.1177/1352458517731914
20. Nato Y, Nagaharu K, Itoh K, Shinke N, Maeyama K, Sawaki A, et al. Autoimmune Hemolytic Anemia Associated with Mature Ovarian Cystic Teratoma Containing Monoclonal Immunoglobulin G: A Case Report and Review of Literature. Case Rep Obstet Gynecol. 27 maja 2024;2024:1–5. doi:10.1155/2024/2223281
21. Ikeguchi R, Shimizu Y, Shimomura A, Suzuki M, Shimoji K, Motohashi T, et al. Paraneoplastic AQP4-IgG–Seropositive Neuromyelitis Optica Spectrum Disorder Associated With Teratoma: A Case Report and Literature Review. Neurol Neuroimmunol Neuroinflammation. września 2021;8(5):e1045. doi:10.1212/NXI.0000000000001045
22. Zhou S, Szczygielski J, Winterhoff B, Mattson J. From intractable hiccups to optic neuritis: paraneoplastic neuromyelitis optica with leptomeningeal carcinomatosis in the setting of immature ovarian teratoma. Gynecol Oncol Rep. grudnia 2025;62:101990. doi:10.1016/j.gore.2025.101990
23. Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. lutego 2013;12(2):157–65. doi:10.1016/S1474-4422(12)70310-1
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2026 Wiktoria Kulczykowska , Sylwia Krzyśkowska, Anna Kozińska, Kamila Krukowska, Martyna Krupa, Laura Krawiec

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Stats
Number of views and downloads: 26
Number of citations: 0