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International Journal of Gynecological... Nov 2019Dysgerminomas are malignant ovarian germ-cell tumors that typically affect young women. Although these tumors have an excellent response to chemotherapy, surgery is an...
BACKGROUND
Dysgerminomas are malignant ovarian germ-cell tumors that typically affect young women. Although these tumors have an excellent response to chemotherapy, surgery is an integral part of primary treatment.
OBJECTIVE
To evaluate outcomes of initial cytoreduction in patients diagnosed with dysgerminomas.
METHODS
Patients who underwent primary cytoreductive surgery for ovarian dysgerminoma between January 1985 and December 2013 were identified and included in the study. A comparison was made between patients who underwent optimal versus sub-optimal cytoreduction. Descriptive, comparative statistics and odds ratios were used to establish an association. Survival curves were performed with the Kaplan-Meier method and compared using a log-rank test. A value of p<0.05 was used to establish a statistical difference.
RESULTS
A total of 180 patients with a histologically confirmed dysgerminoma were included in the analysis. A subsection of 37 patients in stages III/IV were analyzed. The median age at diagnosis was 21 years (IQR 18-26). Histologically, 166 (92.2%) patients had pure dysgerminomas, whereas the rest had mixed histologies. The median tumor size was 18 (IQR 12-22) cm. In all stages, factors associated with optimal cytoreduction, were higher lactate dehydrogenase levels (OR=1.01; p=0.03), higher CA125 levels (OR=1.01; p=0.04), receiving adjuvant chemotherapy (OR=0.22; p<0.01), or undergoing treatment in a specialized institution (OR=12.68; p<0.01). Patients in stages III/IV, initially managed outside our institution were less likely to be taken for cytoreduction (OR=16.88; p=0.013). Other factors, including age (OR=1.02; p=0.39), pelvic lymph-node positivity (OR=2.24; p=0.36), pregnancy during follow-up (OR=0.91: p=0.80), or recurrence of disease (OR=1.93; p=0.23) were found to be similar in both groups. Overall survival was higher in optimally cytoreducted patients (100% vs 95.7%; p=0.032) including all stages, but not if considering only stages III/IV (100% vs 90%, p=0.186); disease-free survival was the same for both groups regardless of stage (94.3% vs 91.1%; p=0.36).
CONCLUSION
Patients with optimal surgeries were most likely to be treated in referral centers. Initial residual disease did not significantly alter recurrence, progression, disease-free survival, or overall survival.
Topics: Adolescent; Adult; Cytoreduction Surgical Procedures; Disease-Free Survival; Dysgerminoma; Female; Humans; Neoplasm Staging; Ovarian Neoplasms; Young Adult
PubMed: 31594835
DOI: 10.1136/ijgc-2019-000632 -
Medicine and Pharmacy Reports Jan 2022Complete gonadal dysgenesis or Swyer syndrome is a rare genetic disorder characterized by 46,XY karyotype and female phenotype with undeveloped streak gonads and high...
BACKGROUND AND AIM
Complete gonadal dysgenesis or Swyer syndrome is a rare genetic disorder characterized by 46,XY karyotype and female phenotype with undeveloped streak gonads and high malignancy risk. The condition usually manifests in teenage and young adults with delayed puberty and primary amenorrhea. The purpose of this study was to investigate the incidence and potential malignant outcomes of complete gonadal dysgenesis in Latvia.
METHODS
37 patients were included in a retrospective study from 1996 to 2016. In fifteen cases, additional patient information was available. Information from medical records was collected on age at the time of diagnosis: anamnesis data, laboratory results, histology of gonads, and treatment.
RESULTS
Complete gonadal dysgenesis with karyotype 46,XY was proven in 36 (97.3%) cases and one (2.7%) case with karyotype 47,XY,+21. The average age of patients at the time of diagnosis was 15.4 ± 8.0 years. The study included 15 cases: eight patients (53.3%) were investigated for primary amenorrhea, and incomplete development of secondary sexual characteristics, 5 patients (33.3%) with abdominal pain and lower abdominal mass, 2 patients (13.3%) were diagnosed at birth. Gonadectomy was performed in 12 cases (80%). The median time between diagnosis and gonadectomy was 0.4 ± 4.3 years. The histopathology results from the gonadal biopsy showed malignancy in 7 cases (58.3%). The most commonly diagnosed tumors were dysgerminoma and gonadoblastoma.
CONCLUSION
Early diagnosis of Swyer syndrome is necessary in view of the risk of malignancy that can develop at a young age. In several cases, the diagnosis of the syndrome was made only after the malignant process development. The study showed the median time between diagnosis and gonadectomy was suboptimal. Therefore, women with amenorrhea and lack of secondary sexual characteristics require careful investigation.
PubMed: 35720238
DOI: 10.15386/mpr-2064 -
Medicine Nov 2020Ovarian dysgerminoma (OD) mostly affect young women, have a rapid growth rate, and could result in complications such as rupture, hemoperitoneum or torsion, and acute...
INTRODUCTION
Ovarian dysgerminoma (OD) mostly affect young women, have a rapid growth rate, and could result in complications such as rupture, hemoperitoneum or torsion, and acute abdomen. However, there have been no reports of OD on F-FDG PET/CT imaging.
PATIENT CONCERNS
A 21-year-old female patient was admitted to our hospital on February 6, 2016, due to "reduced menstrual flow with abdominal distension for 3 months".
DIAGNOSIS
Color Doppler ultrasound showed a large solid mass in the abdomen and pelvis. Serum carbohydrate antigen 125 (CA125) was elevated significantly. Subsequent computed tomography (CT) of chest showed a large effusion in the right thoracic cavity. Abdominal CT scan revealed the presence of a solid mass occupying a large space in the middle and lower abdomen, suggesting that it derived from the left ovary. Then, she underwent F-fluoro-2-deoxy-D-glucose (F-FDG) positron emission tomography (PET)/CT examination for further diagnosis and staging. PET/CT showed a large occupying lesion in the abdomen. The maximum standardized uptake (SUVmax) of F-FDG was 15.8. No obvious hypermetabolic metastases were observed in the other parts of the body. Postoperative pathology and immunohistochemistry confirmed the ovarian dysgerminoma.
INTERVENTIONS
The patient underwent surgery. Chemotherapy was successfully carried out post-operation.
OUTCOMES
Fortunately, the patient is responding well to treatment and the postoperative recurrence-free survival time has been more than 3 years.
CONCLUSION
OD usually occurs in young women and is characterized by large solid pelvic mass. The F-FDG PET/CT scan shows abnormally increased metabolism of the tumor. Because of the high metabolic characteristics, F-FDG PET/CT may be of great significance in the diagnosis and staging of OD.
Topics: Dysgerminoma; Female; Fluorodeoxyglucose F18; Humans; Ovarian Neoplasms; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Young Adult
PubMed: 33157971
DOI: 10.1097/MD.0000000000023074 -
Journal of Pediatric and Adolescent... Feb 2020Girls with Turner syndrome with Y-chromosome material (TS + Y) are assumed to have nonfunctional gonads with increased tumor risk, therefore prophylactic gonadectomy...
STUDY OBJECTIVE
Girls with Turner syndrome with Y-chromosome material (TS + Y) are assumed to have nonfunctional gonads with increased tumor risk, therefore prophylactic gonadectomy is recommended at diagnosis. In this study we aimed to determine rates of spontaneous thelarche (ST) and spontaneous menarche (SM), and prevalence of gonadal tumor and malignancy in girls with TS + Y, to further inform discussions about gonadectomy.
DESIGN
Retrospective review of clinical and pathology data.
SETTING
Multicenter study involving 4 United States children's hospitals.
PARTICIPANTS
Patients included those with a genetically proven diagnosis of TS + Y and phenotypically female genitourinary exam.
INTERVENTIONS
Demographic characteristics, pubertal development, and gonadal pathology data were abstracted from clinical records. Data for ST were analyzed for patients aged 13 years and older and SM for patients older than 15 years.
MAIN OUTCOME MEASURES
ST, SM, prevalence of gonadal tumor, and malignancy.
RESULTS
Forty-four patients met inclusion criteria. Nineteen patients were 13 years or older; 8/19 (42%) had ST and reached Tanner stages 2-4 and 2 (11%) had normal ovarian pathology. Nineteen patients were 15 years or older; 2/19 (11%) had SM. Thirty-seven patients underwent gonadectomy; 35 had available pathology results. Gonadoblastoma was identified in 35/7 patients (19%), 1 in situ germ cell neoplasia, and 1 dysgerminoma (3%). One patient with bilateral gonadoblastoma had ST and SM.
CONCLUSION
In this multicenter cohort, 42% of girls with TS + Y entered puberty spontaneously and 11% had SM, supportive of gonadal function. Risk of tumor was similar to previous reports. To achieve informed decision-making, discussions about gonadectomy should incorporate potential for gonadal function and tumor risk.
Topics: Adolescent; Castration; Child; Chromosomes, Human, Y; Disease Progression; Female; Gonadoblastoma; Gonads; Humans; Menarche; Retrospective Studies; Risk Factors; Turner Syndrome
PubMed: 31465855
DOI: 10.1016/j.jpag.2019.08.011 -
Gynecologic and Obstetric Investigation 2021The aim of this study was to evaluate the long-term outcomes and the factors related to patient prognosis.
OBJECTIVE
The aim of this study was to evaluate the long-term outcomes and the factors related to patient prognosis.
MATERIALS AND METHODS
We retrospectively analyzed patients treated at the Department of Gynecology, Sun Yat-sen University Cancer Center, between January 1, 1968, and December 12, 2018.
RESULTS
A total of 107 patients were identified. Of all patients, 79 (73.8%) presented with stage I disease, 14 (13.1%) stage II, 13 (12.2%) stage III, and 1 (0.9%) stage IV. All patients received surgery, with 70 (65.4%) undergoing fertility-sparing surgery (FS) and 37 (34.6%) nonfertility-sparing surgery (NFS). Ninety patients received postoperative chemotherapy. Nine of the 43 cases with a lymphadenectomy had metastasis (20.9%). The median follow-up time was 132 months (range, 1-536 months). The overall 5-year and 10-year survival was 95.1% and 91.7%, respectively. The 10-year survival rate for stage I and II-IV patients was 96.1% and 79.1%, respectively (p = 0.008). For the patients undergoing FS and NFS, the 10-year disease-free survival rate was 82.3% and 88.0%, respectively (p = 0.403). The 10-year disease-free survival rate for patients with or without lymphadenectomy was 95.1% and 78.4%, respectively (p = 0.040), and it was 92.5% and 76.0%, respectively (p = 0.041), for those with or without omentectomy. Fifteen patients relapsed, and 4 of them (26.7%) had recurrence in the lymph nodes. Eleven of the 15 relapsed patients (73.3%) had been successfully salvaged.
LIMITATIONS
As a study of a rare disease, our analysis was limited by its small sample size and the deemed disadvantage of a retrospective study.
CONCLUSION
Excellent treatment results can be achieved in dysgerminoma patients who received proper treatment. Lymphadenectomy may improve patient survival. Relapsed patients can also be successfully salvaged.
Topics: Dysgerminoma; Humans; Neoplasm Recurrence, Local; Neoplasm Staging; Ovarian Neoplasms; Prognosis; Retrospective Studies
PubMed: 34794146
DOI: 10.1159/000519515 -
Japanese Journal of Clinical Oncology Mar 2020To determine the impact of lymphadenectomy (LND) on survival of clinically apparent early-stage malignant ovarian germ cell tumors (MOGCTs).
OBJECTIVE
To determine the impact of lymphadenectomy (LND) on survival of clinically apparent early-stage malignant ovarian germ cell tumors (MOGCTs).
METHODS
We retrospectively analyzed the survival of patients who were diagnosed with the three most common histopathology types of malignant ovarian germ cell tumors (yolk sac tumor, dysgerminoma and immature teratoma) and with clinical stage I and II disease, and treated at Sun Yat-sen University Cancer Center between 1 January 1970 and 30 September 2018.
RESULTS
There were 227 stage I, 28 stage II and one stage IIIA cases after surgery. One hundred and twenty-six patients underwent lymphadenectomy and 130 did not. Only one lymph node metastasis (0.8%) was found in the lymphadenectomy group. Two hundred and twenty-four out of 256 patients (87.5%) received postoperative chemotherapy. There were five relapses (4.0%) in the lymphadenectomy group and four (3.1%) in the non-lymphadenectomy group. Median follow-up was 68 months (range, 1-388). The 10-year disease-free survival rate in the lymphadenectomy group and non-lymphadenectomy group were 88.2 and 96.4%, respectively (P = 0.412); the 10-year overall survival rate in the two groups were 95.7 and 98.2%; respectively (P = 0.798). The results showed that lymphadenectomy did not improve patient survival.
CONCLUSIONS
Lymphadenectomy may have little impact on survival in patients with clinically apparent early-stage malignant ovarian germ cell tumors and may be omitted in the surgical treatment.
Topics: Adolescent; Adult; Aorta, Abdominal; Child; Child, Preschool; Female; Humans; Lymph Node Excision; Lymphatic Metastasis; Middle Aged; Neoplasm Recurrence, Local; Neoplasm Staging; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Pelvis; Prognosis; Retrospective Studies
PubMed: 31867612
DOI: 10.1093/jjco/hyz180 -
Indian Journal of Surgical Oncology Sep 2022The objective of this study is to analyze the impact of clinicopathological and treatment-related factors on survival in patients with malignant ovarian germ cell tumor....
The objective of this study is to analyze the impact of clinicopathological and treatment-related factors on survival in patients with malignant ovarian germ cell tumor. A total of 253 patients of ovarian germ cell malignancy were retrospectively reviewed during 2000-2019. Out of these, 111 had primary treatment at our institute, which is a dedicated regional cancer center. The remaining 142 were operated elsewhere and were referred to us for adjuvant chemotherapy or with recurrent disease. The clinicopathological and treatment-related characteristics were analyzed for association with tumor persistence/recurrence or death. Among them, 107 were dysgerminomas; 60 had endodermal sinus tumor, 53 mixed germ cell tumors, and 31 immature teratoma; and one each had embryoma and primitive germ cell tumor. The median follow-up period was 19 months (range 0-214). Median time to recurrence or progression was 5 months. Forty-nine patients (19.4%) had a recurrence and there were 16 (6.3%) deaths. Five-year disease-free-survival was 71.3% and 5-year overall survival rate was 88.1%, for the entire cohort. Disease-free-survival was 90.4% and overall survival was 92.1% for patients entirely treated at the reporting institute. Sub-group analysis based on treatment adequacy showed that survival rate was 91.0% in patients who had timely and complete initial treatment versus 78.3% in patients where treatment was incomplete or delayed ( = 0.032). Factors affecting relapse were tumor histology, absence of surgical staging, presence of residual disease, inadequate response to chemotherapy, treatment outside reporting institute, and incomplete/delayed chemotherapy. Significant factors adversely affecting survival were presence of post-operative residual disease, tumor histology, incomplete response to chemotherapy, and inadequate/delayed treatment at primary setting. There was no statistically significant difference based on disease stage and whether fertility-sparing surgery or non-fertility-sparing surgery was performed. Prognosis of ovarian germ cell malignancies is excellent with timely, optimal treatment. The outcome improves significantly if managed adequately in the primary setting, involving dedicated gynecologic oncologists.
PubMed: 36187515
DOI: 10.1007/s13193-022-01537-3 -
Case Reports in Oncology 2021Dysgerminoma is an uncommon malignant tumor arising from the germ cells of the ovary. Its association with pregnancy is extremely rare, with a reported incidence of...
Dysgerminoma is an uncommon malignant tumor arising from the germ cells of the ovary. Its association with pregnancy is extremely rare, with a reported incidence of about 0.2-1 per 100,000 pregnancies. Women in the reproductive age group are more commonly affected. It can be extremely rare to conceive naturally, without assisted reproductive interventions, in cases with ovarian dysgerminoma. If a pregnancy does occur with a concurrent dysgerminoma, it is even more unusual to carry the pregnancy to viability or childbirth without fetal or maternal compromise. We report a case of right ovarian dysgerminoma in a young female with a viable intrauterine pregnancy at 10 weeks, which is rarely diagnosed and managed at this gestational age. Numerous factors played a role in her favorable outcome, including early suspicion by ultrasound and presenting history, surgery, histopathological assessment, imaging, and involvement of the multidisciplinary oncology team. Ovarian neoplasms may rapidly increase in size within a short period with little or no symptoms. This poses a diagnostic challenge for obstetricians and oncologists. Hence, we aimed to evaluate the role of imaging in pregnancy using ultrasound as an imaging modality for both early detection of ovarian neoplasms and for follow-up. In conclusion, patients with ovarian dysgerminoma in pregnancy can have favorable outcomes. Treatment should be individualized on a case-to-case basis, depending on many factors; cancer stage, previous reproductive history, the impact of imaging in staging or follow-up of tumor on the fetus, fetal gestational age, and whether termination of the pregnancy can improve survival or morbidity for the mother.
PubMed: 33776695
DOI: 10.1159/000513622 -
Cancer Imaging : the Official... Oct 2021Imaging findings have a prominent role in early and correct identification of ovarian dysgerminoma, the most common ovarian malignant germ cell tumor (OMGCT). Despite...
BACKGROUND
Imaging findings have a prominent role in early and correct identification of ovarian dysgerminoma, the most common ovarian malignant germ cell tumor (OMGCT). Despite Computed Tomography (CT) is widely used, Magnetic Resonance Imaging (MRI) has proved to be superior in adnexal masses characterization. Limited data and small series are available concerning MRI aspects of dysgerminoma.
CASE PRESENTATION
From January 2012 to December 2018, a database of solid ovarian masses was retrospectively reviewed. Eight patients with histologically proven pure ovarian dysgerminoma and complete imaging available were identified and analyzed. Imaging findings were evaluated separately by two radiologists expert in female genito-urinary MRI.
CONCLUSIONS
MRI findings of a lobulated, purely solid, encapsulated mass with hyper-intensity of lobules and hypo-intensity of septa on T2w images contribute to differentiate dysgerminomas from other ovarian neoplasms.
Topics: Adolescent; Adult; Dysgerminoma; Female; Humans; Magnetic Resonance Imaging; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Retrospective Studies
PubMed: 34711293
DOI: 10.1186/s40644-021-00427-1 -
Frontiers in Pediatrics 2022This retrospective study sought to investigate the risk and proportion of gonadal neoplasms in phenotypic female pediatric patients with DSD and the presence of the Y...
OBJECTIVES
This retrospective study sought to investigate the risk and proportion of gonadal neoplasms in phenotypic female pediatric patients with DSD and the presence of the Y chromosome and different genetic backgrounds in a single Chinese center.
MATERIALS AND METHODS
From January 2012 to December 2020, pediatric and adolescent patients with DSD and the presence of the Y chromosome who had unambiguous female genitalia and underwent bilateral gonadectomy or gonadal biopsy were included in this study. Patients' demographics, karyotype, laboratory test results, gross pathology, and histology of gonadal tissue were all collected. The patients were divided into three groups based on their different genetic backgrounds, and the percentage of gonadal tumors was calculated to assess the risk of gonadal tumor and malignancy by etiology.
RESULTS
A total of 22 patients with DSD and an unambiguous female phenotype with a Y chromosome were recruited. The mean age was 10.91 ± 4.99 years (9 months to 19 years). Gonadal neoplasia was confirmed in six (27.3%) cases by pathological examination of surgical gonadal tissue samples. Among 44 gonadal samples from these 22 patients, the following were identified: five gonadoblastomas, three dysgerminomas, and two Leydig cell tumors. The youngest patient with a tumor was a 2-year-old girl with 46,XY complete gonadal dysgenesis (46,XY CGD or Swyer syndrome) and bilateral gonadoblastoma. Patients with 46,XY complete gonadal dysgenesis (4/6; 66.7%) had the highest tumor occurrence rate. Among 10 patients with Turner syndrome with the presence of the Y chromosome, only one patient was diagnosed with a gonadal tumor. Leydig cell tumor was diagnosed in only one of six patients with 46,XY androgen synthesis/action disorders.
CONCLUSION
Pediatric patients with 46,XY complete gonadal dysgenesis had a significantly increased risk of developing gonadal tumors and underwent prophylactic gonadectomy as soon as the diagnosis was confirmed, whereas those with Turner syndrome with Y chromosome and 46,XY androgen synthesis/action disorders had a relatively low risk. In view of the limited number of patients, a large multicenter study with close follow-ups is needed to support these conclusions.
PubMed: 35935368
DOI: 10.3389/fped.2022.856128