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International Journal of Molecular... Mar 2023Ovarian dermoid cysts, also called mature cystic teratomas (MCTs), account for 69% of ovarian germ cell tumors in young women. The tumors are formed by tissues derived... (Review)
Review
Ovarian dermoid cysts, also called mature cystic teratomas (MCTs), account for 69% of ovarian germ cell tumors in young women. The tumors are formed by tissues derived from three germ layers, and sebaceous materials are most commonly seen. The origin of MCTs is widely considered to be the germ cell origin, which completes meiosis I. The clinical symptoms vary widely, but 20% of tumors could be asymptomatic. The diagnosis of MCTs is usually made without difficulty by ultrasound and confirmed by histopathology post-operatively. The imaging findings have a high diagnostic value. The typical characteristics present in the sonographic images, including a dermoid plug or Rokitansky nodule, are considered strong evidence for a teratoma. Although the malignant transformation of MCTs is rare, it can occur in some cases, especially in women of advanced age. The treatment of MCTs depends on the risk of malignancy, the age of the patient, and the patient's fertility reserve requirement. In this article, we review the epidemiology, clinical symptoms, diagnosis criteria, cellular origin, and treatment of mature cystic teratomas.
Topics: Humans; Female; Teratoma; Ovarian Neoplasms; Meiosis; Cell Transformation, Neoplastic
PubMed: 37047114
DOI: 10.3390/ijms24076141 -
Ultrasound in Obstetrics & Gynecology :... Oct 2022To describe the clinical and ultrasound features of ovarian mature cystic teratomas (MCTs). (Review)
Review
OBJECTIVE
To describe the clinical and ultrasound features of ovarian mature cystic teratomas (MCTs).
METHODS
This was a retrospective study. From the International Ovarian Tumor Analysis (IOTA) database, we identified patients with a histologically confirmed diagnosis of MCT who had undergone transvaginal ultrasound examination between 1999 and 2016 (IOTA phases 1, 2, 3 and 5) in one of five centers. Ultrasound was performed by an experienced examiner who used the standardized IOTA examination technique and terminology. In addition to extracting data from the IOTA database, available two-dimensional grayscale and color or power Doppler images were reviewed retrospectively to identify typical ultrasound features of MCT described previously and detect possible new features using pattern recognition. All images were reviewed by two independent examiners and further discussed with two ultrasound experts to reach consensus.
RESULTS
Included in the study were 454 patients with histologically confirmed MCT. Median age was 33 (range, 8-90) years and 66 (14.5%) patients were postmenopausal. Most MCTs were described by the original ultrasound examiner as unilocular (262/454 (57.7%)) or multilocular (70/454 (15.4%)) cysts with mixed echogenicity of cystic fluid (368/454 (81.1%)), acoustic shadowing (328/454 (72.2%)) and no or little vascularization on color Doppler (color score 1, 240/454 (52.9%); color score 2, 123/454 (27.1%)). The median largest lesion diameter was 66 (range, 15-310) mm. A correct preoperative diagnosis of MCT was suggested by the original ultrasound examiner in 372/454 (81.9%) cases. On retrospective review of ultrasound images of 334 MCTs that had quality sufficient for assessment, 'dots and/or lines' and/or 'echogenic white ball' (typical features according to the literature) were present in 271/334 (81.1%) masses. We identified four new ultrasound features characteristic of MCT: 'cotton wool tufts', 'mushroom cap sign', 'completely hyperechogenic lesion' and 'starry sky sign'. At least one classical or novel ultrasound feature was present in 315/334 (94.3%) MCTs. Twenty-nine (8.7%) MCTs manifested vascularized solid tissue, of which seven exhibited no typical features.
CONCLUSION
We provide a comprehensive overview of conventional and newly described ultrasound features of MCTs. Only a small proportion of MCTs did not manifest any of the typical features. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Adult; Female; Genital Diseases, Female; Humans; Ovarian Neoplasms; Pregnancy; Retrospective Studies; Teratoma; Ultrasonography; Ultrasonography, Doppler, Color
PubMed: 35316568
DOI: 10.1002/uog.24904 -
Annals of Neurology Jan 2007To report the autoantigens of a new category of treatment-responsive paraneoplastic encephalitis. (Clinical Trial)
Clinical Trial Comparative Study
OBJECTIVE
To report the autoantigens of a new category of treatment-responsive paraneoplastic encephalitis.
METHODS
Analysis of clinical features, neuropathological findings, tumors, and serum/cerebrospinal fluid antibodies using rat tissue, neuronal cultures, and HEK293 cells expressing subunits of the N-methyl-D-aspartate receptor (NMDAR).
RESULTS
Twelve women (14-44 years) developed prominent psychiatric symptoms, amnesia, seizures, frequent dyskinesias, autonomic dysfunction, and decreased level of consciousness often requiring ventilatory support. All had serum/cerebrospinal fluid antibodies that predominantly immunolabeled the neuropil of hippocampus/forebrain, in particular the cell surface of hippocampal neurons, and reacted with NR2B (and to a lesser extent NR2A) subunits of the NMDAR. NR2B binds glutamate and forms heteromers (NR1/NR2B or NR1/NR2A/NR2B) that are preferentially expressed in the adult hippocampus/forebrain. Expression of functional heteromers (not single subunits) was required for antibody binding. Eleven patients had teratoma of the ovary (six mature) and one a mature teratoma in the mediastinum; five of five tumors examined contained nervous tissue that strongly expressed NR2 subunits and reacted with patients' antibodies. Tumor resection and immunotherapy resulted in improvement or full recovery of eight of nine patients (paralleled by decreased antibody titers); two of three patients without tumor resection died of neurological deterioration. Autopsies showed extensive microgliosis, rare T-cell infiltrates, and neuronal degeneration predominantly involving, but not restricted to, the hippocampus.
INTERPRETATION
Antibodies to NR2B- and NR2A-containing heteromers of the NMDAR associate with a severe but treatment-responsive encephalitis. Our findings provide a diagnostic test and suggest a model of autoimmune NMDAR-related encephalitis with broad implications for other immune-mediated disorders of memory, behavior, and cognition.
Topics: Adolescent; Adult; Animals; Antibodies; Dissection; Encephalitis; Female; Gene Expression Regulation, Neoplastic; Humans; Immunohistochemistry; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Neurons; Ovarian Neoplasms; Protein Subunits; Rats; Rats, Wistar; Receptors, N-Methyl-D-Aspartate; Teratoma; Transfection; Treatment Outcome
PubMed: 17262855
DOI: 10.1002/ana.21050 -
Medical Hypotheses Sep 2017Spontaneous parthenogenetic and androgenetic events occur in humans, but they result in tumours: the ovarian teratoma and the hydatidiform mole, respectively. However,...
Spontaneous parthenogenetic and androgenetic events occur in humans, but they result in tumours: the ovarian teratoma and the hydatidiform mole, respectively. However, the observation of fetiform (ovarian) teratomas, the serependious identification of several chimeric human parthenotes and androgenotes in the last two decades, along with the creation of viable bi-maternal mice in the laboratory based on minor genetic interferences, raises the question of whether natural cases of clinically healthy human parthenotes have gone unnoticed to science. Here we present a hypothesis based on three elements to support the existence of such elusive individuals: mutations affecting (i) genomic imprinting, (ii) meiosis and (iii) oocyte activation. Additionally, we suggest that the routine practice of whole genome sequencing on every single newborn worldwide will be the ultimate test to this controversial, yet astonishing hypothesis. Finally, several medical implications of such intriguing event are presented.
Topics: Animals; Chimera; Diploidy; Female; Genomic Imprinting; Heterozygote; Humans; Meiosis; Mice; Models, Genetic; Mutation; Oocytes; Ovarian Neoplasms; Parthenogenesis; Teratoma; Whole Genome Sequencing
PubMed: 28818272
DOI: 10.1016/j.mehy.2017.07.008 -
Brain : a Journal of Neurology Aug 2022Autoantibodies against the extracellular domain of the N-methyl-d-aspartate receptor (NMDAR) NR1 subunit cause a severe and common form of encephalitis. To better...
Autoantibodies against the extracellular domain of the N-methyl-d-aspartate receptor (NMDAR) NR1 subunit cause a severe and common form of encephalitis. To better understand their generation, we aimed to characterize and identify human germinal centres actively participating in NMDAR-specific autoimmunization by sampling patient blood, CSF, ovarian teratoma tissue and, directly from the putative site of human CNS lymphatic drainage, cervical lymph nodes. From serum, both NR1-IgA and NR1-IgM were detected more frequently in NMDAR-antibody encephalitis patients versus controls (both P < 0.0001). Within patients, ovarian teratoma status was associated with a higher frequency of NR1-IgA positivity in serum (OR = 3.1; P < 0.0001) and CSF (OR = 3.8, P = 0.047), particularly early in disease and before ovarian teratoma resection. Consistent with this immunoglobulin class bias, ovarian teratoma samples showed intratumoral production of both NR1-IgG and NR1-IgA and, by single cell RNA sequencing, contained expanded highly-mutated IgA clones with an ovarian teratoma-restricted B cell population. Multiplex histology suggested tertiary lymphoid architectures in ovarian teratomas with dense B cell foci expressing the germinal centre marker BCL6, CD21+ follicular dendritic cells, and the NR1 subunit, alongside lymphatic vessels and high endothelial vasculature. Cultured teratoma explants and dissociated intratumoral B cells secreted NR1-IgGs in culture. Hence, ovarian teratomas showed structural and functional evidence of NR1-specific germinal centres. On exploring classical secondary lymphoid organs, B cells cultured from cervical lymph nodes of patients with NMDAR-antibody encephalitis produced NR1-IgG in 3/7 cultures, from patients with the highest serum NR1-IgG levels (P < 0.05). By contrast, NR1-IgG secretion was observed neither from cervical lymph nodes in disease controls nor in patients with adequately resected ovarian teratomas. Our multimodal evaluations provide convergent anatomical and functional evidence of NMDAR-autoantibody production from active germinal centres within both intratumoral tertiary lymphoid structures and traditional secondary lymphoid organs, the cervical lymph nodes. Furthermore, we develop a cervical lymph node sampling protocol that can be used to directly explore immune activity in health and disease at this emerging neuroimmune interface.
Topics: Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Autoantibodies; Female; Germinal Center; Humans; Immunoglobulin A; Immunoglobulin G; Lymphatic Vessels; Ovarian Neoplasms; Receptors, N-Methyl-D-Aspartate; Teratoma
PubMed: 35680425
DOI: 10.1093/brain/awac088 -
International Journal of Molecular... Oct 2021Ovarian teratomas are by far the most common ovarian germ cell tumor. Most teratomas are benign unless a somatic transformation occurs. The designation of teratoma... (Review)
Review
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.
Topics: Animals; Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Autoantibodies; Disease Models, Animal; Female; Humans; Ovarian Neoplasms; Signal Transduction; Teratoma
PubMed: 34681570
DOI: 10.3390/ijms222010911 -
BMJ Case Reports May 2020A 9-year-old girl attended the emergency department with right-sided abdominal pain and vomiting. Due to history and following examination, an ultrasound was requested... (Review)
Review
A 9-year-old girl attended the emergency department with right-sided abdominal pain and vomiting. Due to history and following examination, an ultrasound was requested which demonstrated a large complex midline mass. The most likely diagnosis was ovarian torsion, for which the patient underwent laparoscopy, detorsion and ovarian cystectomy. Histology revealed a mature cystic teratoma. Although less common than in the adult population, it is important to consider ovarian torsion in children and adolescents. Presentation is usually with pain accompanied by vomiting and fever, although these symptoms are not always present. Current management is organ-sparing, with laparoscopy±cystectomy. We discuss the adaptations for this procedure with regard to the paediatric and adolescent population. Differences in the anatomy and physiology must lead to consideration for alterations in surgical technique and positioning to ensure the safest and best quality care for these young patients.
Topics: Child; Diagnosis, Differential; Female; Humans; Laparoscopy; Ovarian Neoplasms; Ovarian Torsion; Teratoma
PubMed: 32404320
DOI: 10.1136/bcr-2019-232610 -
BMC Veterinary Research Nov 2020Teratomas are germ cell neoplasms composed of a wide variety of tissues. In the woodchuck, only one testicular teratoma has been described in the literature. The...
BACKGROUND
Teratomas are germ cell neoplasms composed of a wide variety of tissues. In the woodchuck, only one testicular teratoma has been described in the literature. The objective of this report was to describe the radiologic and pathologic findings in a female woodchuck (Marmota monax) with an ovarian teratoma consisting of mature tissues originating from all three germ layers.
CASE PRESENTATION
A 2-year-old female woodchuck that had been infected at birth with woodchuck hepatitis virus and subsequently developed hepatocellular carcinoma was incidentally discovered to have a mobile 6.6 × 4.8 × 4.7 cm abdominal mass on computed tomography (CT) imaging. The tumor was predominantly solid and heterogenous on CT with soft tissue, fat, and areas of dense calcification. The teratoma did not enhance with intravenous contrast administration. On ultrasound, the tumor was solid with heterogeneous echogenicity, reflecting the fat content and areas of calcification. Sonolucent areas were present that may have represented cysts. There was heterogeneously increased signal on T1-weighted magnetic resonance imaging (MRI) and heterogeneous hyperintensity in T2-weighted imaging. Fat was evident within the tumor. At necropsy, the tumor was attached to the distal end of the right uterine horn. Histopathology showed mature tissue types representing all three germ layers.
CONCLUSIONS
Ovarian teratoma should be considered in the differential diagnosis of ovarian or abdominal masses in woodchucks. The tumor displayed mature tissue derived from all three germ layers. CT, ultrasound, and MRI findings were presented in detail and matched the typical imaging appearance of teratomas.
Topics: Animals; Carcinoma, Hepatocellular; Female; Hepatitis B; Hepatitis B Virus, Woodchuck; Liver Neoplasms; Magnetic Resonance Imaging; Marmota; Ovarian Neoplasms; Teratoma; Tomography, X-Ray Computed; Ultrasonography
PubMed: 33228678
DOI: 10.1186/s12917-020-02658-z -
Annals of Clinical and Translational... Jan 2024Ovarian teratoma is a common occurrence in patients with anti-NMDA receptor encephalitis (NMDARe), and its removal is crucial for a favorable prognosis. However, the...
INTRODUCTION
Ovarian teratoma is a common occurrence in patients with anti-NMDA receptor encephalitis (NMDARe), and its removal is crucial for a favorable prognosis. However, the initial pathogenesis of autoimmunity in the encephalitic teratoma remains unclear. In this study, we aimed to investigate the genomic landscape and microscopic findings by comparing NMDARe-associated teratomas and non-encephalitic control teratomas.
MATERIALS AND METHODS
A prospective consecutive cohort of 84 patients with NMDARe was recruited from January 2014 to April 2020, and among them, patients who received teratoma removal surgery at Seoul National University Hospital were enrolled. We conducted a comparison of whole-exome sequencing data and pathologic findings between NMDARe-associated teratomas and control teratomas.
RESULTS
We found 18 NMDARe-associated teratomas from 15 patients and compared them with 17 non-encephalitic control teratomas. Interestingly, the genomic analysis revealed no significant differences in mutations between encephalitic and non-encephalitic teratomas. Pathologic analysis showed no discrepancies in terms of the presence of neuronal tissue and lymphocytic infiltration between the encephalitic teratomas (n = 14) and non-encephalitic teratomas (n = 18). However, rituximab-naïve encephalitic teratomas exhibited a higher frequency of germinal center formation compared to non-encephalitic teratomas (80% vs. 16.7%, P = 0.017). Additionally, rituximab-treated encephalitic teratomas demonstrated a reduced number of CD20 cells and germinal centers in comparison to rituximab-naïve encephalitic teratomas (P = 0.048 and 0.023, respectively).
DISCUSSION
These results suggest that the initiation of immunopathogenesis in NMDARe-associated teratoma is not primarily attributed to intrinsic tumor mutations, but rather to immune factors present in the encephalitic patient group, ultimately leading to germinal center formation within the teratoma.
Topics: Female; Humans; Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Rituximab; Prospective Studies; Ovarian Neoplasms; Teratoma; Receptors, N-Methyl-D-Aspartate; Genomics
PubMed: 37986706
DOI: 10.1002/acn3.51948 -
Journal of the Chinese Medical... Jun 2018
Topics: Adolescent; Biomarkers, Tumor; Female; Humans; Ovarian Neoplasms; Teratoma
PubMed: 28882729
DOI: 10.1016/j.jcma.2017.08.002