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Modern Pathology : An Official Journal... Feb 2021Mature ovarian teratoma is considered to be a parthenogenetic tumor that arises from a single oocyte/ovum. Conversely, complete hydatidiform mole (CHM) is androgenetic...
Mature ovarian teratoma is considered to be a parthenogenetic tumor that arises from a single oocyte/ovum. Conversely, complete hydatidiform mole (CHM) is androgenetic in origin: classic CHM arises from a single or two sperm. Since mature ovarian teratoma and CHM have only maternal and paternal genomes, respectively, their genome imprinting is theoretically reverse, but this has yet to be investigated. Genome imprinting in struma ovarii, a special form of mature teratoma, remains unclear. Although a mature teratoma can rarely arise in extragonadal sites, its genome imprinting, as well as cell origin, is poorly understood. One of the most important mechanisms of genome imprinting is DNA methylation. To investigate the methylation profile of imprinted genes, we performed methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) of 21 imprinting control region (ICRs) of 9 imprinted genes/gene clusters in formalin-fixed, paraffin-embedded samples obtained from 12 mature ovarian teratomas, 6 struma ovarii, 10 CHMs, and 7 extragonadal (1 sacrococcygeal, 6 mediastinal) mature teratomas of females. In mature ovarian teratomas, ICRs of maternally and paternally imprinted genes showed high and low levels of methylation, respectively, and this pattern was almost reverse in CHMs. In CHMs, however, some ICRs showed aberrant methylation. The methylation profile of struma ovarii was comparable to that of mature ovarian teratomas, except for an adenomatous tumor. In extragonadal mature teratomas, the methylation pattern was somatic or irregular. In conclusion, mature ovarian teratomas/struma ovarii, CHMs, and extragonadal mature teratomas showed distinct methylation profiles of imprinted genes. Ovarian teratomas and CHMs are most likely to inherit their methylation profiles from their ancestral germ cells, although some aberrant methylation suggests a relaxation of imprinting in CHMs and a subset of struma ovarii. Extragonadal mature teratomas may carry a methylation profile of misplaced primordial germ cells or possibly somatic cells that have been reprogrammed in vivo.
Topics: DNA Methylation; Female; Genomic Imprinting; Humans; Hydatidiform Mole; Ovarian Neoplasms; Pregnancy; Teratoma; Uterine Neoplasms
PubMed: 32873866
DOI: 10.1038/s41379-020-00668-8 -
Open Access Macedonian Journal of... Mar 2018Ovarian strumal carcinoid is a germ cell tumour characterised by a mixture of thyroid tissue and carcinoid. Ovarian struma is a very rare occurrence with 0.3-1%...
BACKGROUND
Ovarian strumal carcinoid is a germ cell tumour characterised by a mixture of thyroid tissue and carcinoid. Ovarian struma is a very rare occurrence with 0.3-1% incidence of all ovarian tumours and 3% of mature teratomas. Primary carcinoid ovarian tumours are still uncommon as a part of mature teratoma or mucinous cystadenoma. There are four major variants of a carcinoid tumour: insular, trabecular, strumal and mucinous. A strumal carcinoid is an unusual form of ovarian teratoma composed of an intimate admixture of thyroid/carcinoid tissues.
CASE REPORT
This is a case report of a 59-year old woman with a 5-year clinical history of perimenopausal uterine bleeding and three explorative curettages. Gynaecological and ultrasound examinations revealed ovarian enlargement with a diameter of 50 mm with hypoechoic zones suspected of benign teratoma. The diagnostic test such as Ca-125, AFP, free-T4 and TSH was in normal range. A smooth, solid right ovarian 50 an mm-size tumour, as well as small amount of fluid in the Douglas pouch, was found during the total abdominal hysterectomy, bilateral salpingo-oophorectomy and staging biopsy. The histopathology revealed teratoma with strumal carcinoid tumour IA stage according to AJCC 2010 of the right ovary and negative cytopathology of the fluid from the Douglas pouch. On the postoperative 2-year control, the patient was tumour free, and Ca-125, free-T4 and TSH were in normal range.
CONCLUSION
We would like to point out those specific diagnostic tools, such as ultrasound and Ca-125 have low specificity and sensitivity in detection of this rare ovarian malignancy.
PubMed: 29610616
DOI: 10.3889/oamjms.2018.138 -
BMC Women's Health Mar 2022Primary ovarian carcinoid is a very rare ovarian low-grade neuroendocrine tumor, accounting for about 0.1% of all ovarian neoplasms.
BACKGROUND
Primary ovarian carcinoid is a very rare ovarian low-grade neuroendocrine tumor, accounting for about 0.1% of all ovarian neoplasms.
CASE PRESENTATION
We reported a case of primary ovarian carcinoid arising from a mature cystic teratoma in a 50-year-old woman. Intraoperative frozen section of left ovarian mass was assessed and a malignant epithelial tumor was considered. Morphologically, the main tumor was composed of cells forming trabeculae, and mature cystic teratoma was observed adjacent to the main part. Immunohistochemistry revealed that the trabecular cells were diffuse positive for pan Cytokeratin, CD56 and synaptophysin with low Ki-67 index (about 1%).
CONCLUSIONS
Careful morphological observation combined with appropriate accessory examination are essential for the diagnosis of primary ovarian carcinoid arising from mature cystic teratoma. In addition, the classification criteria of the primary ovarian neuroendocrine tumor are discussed.
Topics: Carcinoid Tumor; Female; Humans; Intestinal Neoplasms; Middle Aged; Neuroendocrine Tumors; Ovarian Neoplasms; Pancreatic Neoplasms; Stomach Neoplasms; Teratoma
PubMed: 35300659
DOI: 10.1186/s12905-022-01660-x -
Journal of Ovarian Research Jul 2016Gliomatosis peritonei (GP) is a rare condition characterized by mature glial tissue implants widespread in the peritoneum. The GP is often associated with ovarian... (Review)
Review
BACKGROUND
Gliomatosis peritonei (GP) is a rare condition characterized by mature glial tissue implants widespread in the peritoneum. The GP is often associated with ovarian teratoma. However, little is known about the characteristics and prognosis of GP. The purpose of this study was to describe the features, treatment, and prognosis of GP. Additionally, we review previously reported cases of GP, summarizing the presently known data.
METHODS
From January 2000 to January 2016, cases of ovarian teratoma and GP treated at Peking Union Medical College Hospital were reviewed. We assessed the pathology, treatments, and outcomes along with prognostic information. Additionally, the literature regarding this clinical condition was also reviewed.
RESULTS
Eight patients met the inclusion criteria. Patients had a median age of 20 (range, 15-25) years. GP was diagnosed as the primary tumor in 6 patients and at a secondary surgery in two patients. The primary ovarian tumor consisted of immature teratoma (n = 7) and mature teratoma (n = 1). Grades of immature ovarian teratoma were 2, grade 1; 3, grade 2; and 2, grade 3. Tumors mean had a size of 20.4 (range, 11-30) cm. The median follow-up time was 60.5 (range, 3-144) months. All cases had conservative surgery and seven of them had macroscopic residual disease postoperatively. During the study period, the eight patients remained alive and asymptomatic. Three patients in the study experienced spontaneous pregnancy. After reviewing the existing literature, a total of 14 patients with nodal gliomatosis were present and 10 of them were alive. According to the literature review, five articles reported more than five cases. Of a total of 67 patients, 60 of them remained alive.
CONCLUSION
The prognosis of immature ovarian teratoma with GP is favorable. Complete resection of GP is often difficult. Residual peritoneal disease in GP can be asymptomatic and quiescent over a long period. A more conservative surgical approach may be carried out in patients with massive peritoneal spread after the presence of metastatic immature elements is excluded. Owing to the risk of recurrence and malignant transformation of GP, a long-term follow-up is necessary for patients with residual peritoneal disease.
Topics: Adolescent; Adult; Biomarkers; Combined Modality Therapy; Female; Glioma; Humans; Neoplasm Metastasis; Ovarian Neoplasms; Peritoneal Neoplasms; Pregnancy; Prognosis; Teratoma; Treatment Outcome; Tumor Burden; Young Adult
PubMed: 27473411
DOI: 10.1186/s13048-016-0256-5 -
Journal of Clinical Laboratory Analysis May 2022Teratoma with nephroblastoma (TWN) is an extremely rare condition. Since 1984, only 45 reported cases have been identified. To our knowledge, there have been only two...
BACKGROUND
Teratoma with nephroblastoma (TWN) is an extremely rare condition. Since 1984, only 45 reported cases have been identified. To our knowledge, there have been only two cases of TWN of ovarian origin.
CASE PRESENTATION
We described a case of ovarian TWN who presented to us with painless abdominal masses 6 months after undergoing right ovarian cystectomy. The tumor had spread to the abdomen due to spontaneous rupture of the ovarian cyst and failure to undergo chemotherapy. Microscopically, the ovarian mass exhibited the typical components of a mature cystic teratoma. The tumors found in both the ovary and abdomen contained the nephroblastoma components and were strongly positive for WT-1. The patient was advised to undergo chemotherapy and she was lost to follow-up.
CONCLUSION
A careful histological examination is necessary for an accurate diagnosis, which is based on morphology and extensive immunohistochemical studies. According to the literature, surgical excision alone seems reasonable as the prognosis of TWN is considered to be good. However, due to the spontaneous rupture of the ovarian cyst, chemotherapy of the patient after the first surgery was necessary in our case. Therefore, additional case studies are needed to clarify the standardized treatment of TWN.
Topics: Abdomen; Chest Pain; Female; Humans; Kidney Neoplasms; Ovarian Cysts; Ovarian Neoplasms; Rupture, Spontaneous; Teratoma; Wilms Tumor
PubMed: 35349728
DOI: 10.1002/jcla.24364 -
Gynecologic Oncology Reports Feb 2017Mature cystic teratomas (MCT), also known as dermoid cysts, are the most common ovarian germ cell tumors and the most common ovarian neoplasms in patients younger than... (Review)
Review
Mature cystic teratomas (MCT), also known as dermoid cysts, are the most common ovarian germ cell tumors and the most common ovarian neoplasms in patients younger than 20 years. Malignant transformation (MT) is a rare complication of MCTs which may occur in 1-2% of the cases. Squamous cell carcinoma (SCC) is the most frequent histology arising from MCTs and its appearance depends on diverse risk factors such as patient's age, the size of the tumor and levels of serum tumor markers. Diagnosis and treatment constitute a big challenge due to the rarity and the aggressive course of this entity. Adjuvant chemotherapy has a leading role in the treatment of MCT-arising SCC, while the use of radiotherapy or chemoradiation is still under consideration. Herein, we report a case of a post-menopausal woman, presenting with mild symptoms and a large pelvic mass deriving from the left ovary occurring as dermoid cyst. Simultaneously, we review the literature stressing out the prognostic factors and the treatment options for MCT arising SCC according to traditional and new therapy-strategies.
PubMed: 28050596
DOI: 10.1016/j.gore.2016.12.005 -
Gynecologic Oncology Reports Apr 2022To compare the survival outcomes between surveillance and adjuvant chemotherapy in patients with stage I ovarian immature teratomas (ITs) who underwent fertility sparing...
OBJECTIVE
To compare the survival outcomes between surveillance and adjuvant chemotherapy in patients with stage I ovarian immature teratomas (ITs) who underwent fertility sparing surgery.
METHODS
In this retrospective cohort analysis, patients with stage IA Grade 2-3, stage IB and IC ovarian ITs who underwent surveillance after surgery between 2016 and 2021 from Peking Union Medical College Hospital were identified. A 1:1 propensity score matching was performed in patients who had adjuvant chemotherapy by age, stage, and grade.
RESULTS
A total of 16 patients without adjuvant chemotherapy were identified. There were 3 stage IA and 13 stage IC patients. The median age at diagnosis was 14.5 years old (range 3-30). There were 6 patients who had grade 1 tumor, 5 grade 2, 5 grade 3. After a mean follow-up period of 27.4 months, a patient with stage IA grade 2 IT who underwent ovarian cystectomy had recurrence in the same ovary. 16 patients who received adjuvant chemotherapy were screened as controls. The tumor stage and grade were similar in two groups. The median age was older in the chemotherapy group. Of the 16 matched patients there was one recurrence. There was no statistical difference in 3-year disease-free survival (DFS) between surveillance group and chemotherapy group ( = 0.808).
CONCLUSION
We did not observe survival differences or recurrence rates between patients who underwent adjuvant chemotherapy or those who did not with stage I ovarian ITs. Our study suggests surveillance may be safe and preferable in early-stage IT patients who underwent complete tumor resection.
PubMed: 35434235
DOI: 10.1016/j.gore.2022.100976 -
Acta Neuropathologica Communications Mar 2019Encephalitis with anti-NMDAR antibodies (NMDAR-E) is a severe autoimmune neurological disorder, defined by a clinical presentation of encephalitis and the presence of...
Encephalitis with anti-NMDAR antibodies (NMDAR-E) is a severe autoimmune neurological disorder, defined by a clinical presentation of encephalitis and the presence of IgG targeting the GluN1 subunit of NMDA receptors in the CSF. An underlying ovarian teratoma is commonly associated with this autoimmune disease suggesting a role of the tumor in immunopathogenesis. In this study, we characterized the salient histopathological features of 27 ovarian teratomas associated with NMDAR-E (3 immature and 24 mature teratomas) and 40 controls without associated encephalitis. All but one NMDAR-E-associated teratomas contained a nervous tissue component, while less than 40% of control teratomas did (p < 0.001). GluN1 expression by teratomatous nervous tissue seemed to be more often glial in NMDAR-E teratomas than in control teratomas (73% vs. 29%, p < 0.05). Strikingly, 3 out of 24 NMDAR-E-associated mature teratomas contained neuroglial tissue exhibiting histopathological features of central nervous system neuroglial tumor, while such glioma-like features are exceptionally described in the literature on ovarian teratomas. Moreover, NMDAR-E associated teratomas differed from sporadic ovarian teratomas by consistent and prominent infiltration of the nervous tissue component by immune cells, comprised of T- and B-cells and mature dendritic cells organized in tertiary lymphoid structures, with IgG and IgA deposits and plasma cells in close contact to the neuroglial tissue.These data demonstrate an association between massive infiltration of NMDAR-E-associated teratomas by immune cells and particular glial features of its neuroglial component, suggesting that this glial tissue might be involved in triggering or sustaining the anti-tumor response associated with the auto-immune neurological disease.
Topics: Anti-N-Methyl-D-Aspartate Receptor Encephalitis; B-Lymphocytes; Brain; Female; Humans; Neoplasm Grading; Ovarian Neoplasms; T-Lymphocytes; Teratoma
PubMed: 30857565
DOI: 10.1186/s40478-019-0693-7 -
Acta Obstetricia Et Gynecologica... Jul 2023Primary ovarian carcinoids are extremely rare ovarian tumors, and there is limited data available on their clinical characteristics and survival outcomes.
INTRODUCTION
Primary ovarian carcinoids are extremely rare ovarian tumors, and there is limited data available on their clinical characteristics and survival outcomes.
MATERIAL AND METHODS
We conducted a historical cohort study of 56 patients to investigate their clinical characteristics. The overall survival, disease-specific survival, recurrence-free survival, and potential prognostic factors of these patients were also evaluated.
RESULTS
The median age of these patients was 42.0 years (range: 20-71). The average mass and carcinoid size was 7.3 and 0.4 cm, respectively. Elevated tumor marker levels and ascites were observed in 15 and 10 patients, respectively. In 98.2% of the patients, tumors were confined to the ovary, while only one had metastatic disease. Surgery was the mainstay therapy: 37.5% of the patients underwent unilateral salpingo-oophorectomy, 25.0% underwent hysterectomy with bilateral salpingo-oophorectomy, 21.4% underwent ovarian cystectomy, 10.7% underwent comprehensive staging surgery, and 5.4% underwent bilateral salpingo-oophorectomy. Appendectomy and lymphadenectomy were performed in eight and five patients, respectively, but none showed tumor involvement. Chemotherapy was the only adjuvant treatment utilized, and was administered in four patients. Pathological analysis showed that strumal carcinoid was the most predominant subtype, occurring in 66.1% of the patients. The Ki-67 index was reported in 39 patients, 30 of which had an index of no more than 3%, with a maximum of only 5%. Only one relapse occurred after the initial treatment, and that patient experienced recurrences on two occasions, maintaining stable disease after surgery and octreotide therapy. After a median follow-up of 3.6 years, 96.4% of the patients achieved no evidence of disease, while 3.6% were alive with the disease. The 5-year recurrence-free survival rate was 97.9% and no death occurred. No risk factors for recurrence-free survival, overall survival, or disease-specific survival were identified.
CONCLUSIONS
The Ki-67 indices were extremely low and prognoses were excellent in patients with primary ovarian carcinoids. Conservative surgery, especially unilateral salpingo-oophorectomy, is preferred. Individualized adjuvant therapy may be considered for patients with metastatic diseases.
Topics: Female; Humans; Young Adult; Adult; Middle Aged; Aged; Ovarian Neoplasms; Cohort Studies; Carcinoid Tumor; Ki-67 Antigen; Struma Ovarii; Neoplasm Recurrence, Local; Neoplasm Staging; Retrospective Studies
PubMed: 37059424
DOI: 10.1111/aogs.14578 -
The Journal of International Medical... Aug 2021To investigate the clinical characteristics and treatment of large ovarian masses in adolescents.
OBJECTIVE
To investigate the clinical characteristics and treatment of large ovarian masses in adolescents.
METHODS
Adolescents with large ovarian masses (≥10 cm in diameter) who were treated in Beijing Obstetrics and Gynecology Hospital from March 2010 to December 2018 were retrospectively assessed.
RESULTS
Fifty-two female patients (mean age: 16.17±2.04 years [11-19 years]) were included and 19 (36.5%) presented with abdominal pain. The blood flow signal rate in ultrasonography was significantly different among benign, borderline and malignant ovarian masses, unlike strong echo, dotted echo and septation rates. Carbohydrate antigen 125 positivity rates were significantly different among pathological types and the endometriotic cyst group showed the highest value (75.0%). Alpha-fetoprotein positivity rates were also different among pathological types. For ovarian cystectomy, 14 and 32 patients underwent laparotomy and laparoscopy, respectively. Mass diameters were significantly higher in the laparotomy group and the operative duration was significantly shorter in the laparoscopy group. There were no significant differences in intraoperative blood loss or postoperative recurrence rates between the two groups.
CONCLUSION
Teratomas constitute the greatest group of large ovarian masses in adolescents. Benign tumors should be treated by laparoscopic resection, while borderline or malignant tumors require individualized treatment of tumors and fertility-sparing treatments.
Topics: Adolescent; Female; Humans; Laparoscopy; Laparotomy; Neoplasm Recurrence, Local; Ovarian Neoplasms; Retrospective Studies; Teratoma
PubMed: 34340578
DOI: 10.1177/03000605211032781