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Diagnostics (Basel, Switzerland) Dec 2022Dysgerminoma represents a rare malignant tumor composed of germ cells, originally from the embryonic gonads. Regarding its incidence, we do not have precise data due to... (Review)
Review
Dysgerminoma represents a rare malignant tumor composed of germ cells, originally from the embryonic gonads. Regarding its incidence, we do not have precise data due to its rarity. Dysgerminoma occurs at a fertile age. The preferred treatment is the surgical removal of the tumor succeeded by the preservation of fertility. Even if a multidisciplinary team, founded in 2009 by a gynecologist, an oncologist, a pediatric oncologist and a pediatric surgeon, under the guidance of the Malignant Germ Cell International Consortium (MaGIC), studies this type of tumor, issues still remain related to the lack of a randomized study and to both the management and understanding of the concept of OMGCTs (ovarian malignant germ cell tumors). The aim of this review is to present from the literature the various approaches for this type of tumor, and, regarding innovative therapies or possible prevention, which can be applied in clinical practice. Multidisciplinarity and treatment in reference centers have proven their usefulness as well.
PubMed: 36553112
DOI: 10.3390/diagnostics12123105 -
Abdominal Radiology (New York) May 2023Adnexal masses during pregnancy are a relatively uncommon entity. Their clinical management is challenging given the overlapping features of certain entities on imaging... (Review)
Review
Adnexal masses during pregnancy are a relatively uncommon entity. Their clinical management is challenging given the overlapping features of certain entities on imaging and histopathology, which can mimic malignancy, and the potential side effects to the mother and fetus, whether expectant management versus surgery is pursued. Ultrasonography with Doppler evaluation is the modality of choice for evaluating adnexal masses during pregnancy. Magnetic resonance imaging is the second-line modality useful when US findings are inconclusive/indeterminate. Most adnexal masses in pregnant patients are benign in origin (e.g., functional cysts, mature cystic teratoma, decidualization of endometrioma), but a few are malignant in origin (e.g., dysgerminoma, granulosa cell tumor). Most cases of adnexal masses are asymptomatic, but complications such as ovarian torsion can occur. This review aims to familiarize the radiologist with the imaging of adnexal lesions during pregnancy so that the radiologist can identify ovarian cancer. Specifically, the review will detail the most common benign and malignant adnexal masses in pregnancy, mimickers, and their corresponding imaging findings on US and MRI.
Topics: Pregnancy; Humans; Female; Ovarian Neoplasms; Adnexal Diseases; Magnetic Resonance Imaging; Granulosa Cell Tumor; Dermoid Cyst
PubMed: 36538079
DOI: 10.1007/s00261-022-03768-y -
Polish Journal of Pathology : Official... 2022Terminal deoxynucleotidyl transferase (TdT) is a unique type of DNA polymerase predominantly expressed in precursor lymphoid cells and acute lymphoblastic leukemia. It...
Terminal deoxynucleotidyl transferase (TdT) is a unique type of DNA polymerase predominantly expressed in precursor lymphoid cells and acute lymphoblastic leukemia. It participates in the junctional diversity of T-cell receptors and immunoglobulins. Recently, aberrant TdT expression was found in seminomas. Here, we evaluated the expression of TdT in our cohort of germ cell tumors (GCTs) with two anti-TdT antibody clones. We included 173 cases of testicular GCTs, 5 ovarian dysgerminomas, and one gonadoblastoma in the study. Tissue microarrays containing representative tumor samples were constructed and subsequently stained with anti-TdT monoclonal rabbit antibody EP266 (Dako) and TdT rabbit polyclonal antibody (Cell Marque). Expression was assessed with the H-score. No specific nuclear reaction was observed for the polyclonal anti-TdT antibody. The H-score values varied between the histological subtypes for the EP266 antibody. Positive nuclear staining was consistently seen in germ cell neoplasia in situ , seminoma, dysgerminoma, and embryonal carcinoma. Pure tumors had higher TdT H-scores than the mixed ones. Teratomas, yolk sac tumors, and choriocarcinomas were almost uniformly negative. Our study confirms that aberrant expression of TdT by testicular and ovarian GCTs exemplifies a potential diagnostic pitfall in histopathological diagnostics.
Topics: Humans; Male; Female; Animals; Rabbits; DNA Nucleotidylexotransferase; Immunohistochemistry; Biomarkers, Tumor; Neoplasms, Germ Cell and Embryonal; Testicular Neoplasms; Dysgerminoma; Ovarian Neoplasms; DNA-Directed DNA Polymerase
PubMed: 36345953
DOI: 10.5114/pjp.2022.120098 -
World Journal of Clinical Oncology Oct 2022Malignant ovarian germ cell tumors (MOGCT) are rare and frequently occur in women of young and reproductive age and the oncologic and reproductive outcomes after...
BACKGROUND
Malignant ovarian germ cell tumors (MOGCT) are rare and frequently occur in women of young and reproductive age and the oncologic and reproductive outcomes after fertility-sparing surgery (FSS) for this disease are still limited.
AIM
To evaluate the oncology and reproductive outcomes of MOGCT patients who underwent FSS.
METHODS
All MOGCT patients who underwent FSS defined as the operation with a preserved uterus and at least one side of the ovary at our institute between January 2005 and December 2020 were retrospectively reviewed.
RESULTS
Sixty-two patients were recruited for this study. The median age was 22 years old and over 77% were nulliparous. The three most common histology findings were immature teratoma (32.2%), dysgerminoma (24.2%), and yolk sac tumor (24.2%). The distribution of stage was as follows; Stage I, 74.8%; stage II, 9.7%; stage III, 11.3%; and stage IV, 4.8%. Forty-three (67.7%) patients received adjuvant chemotherapy. With a median follow-up time of 96.3 mo, the 10-year progression-free survival and overall survival were 82.4% and 91%, respectively. For reproductive outcomes, of 43 patients who received adjuvant chemotherapy, 18 (41.9%) had normal menstruation, and 17 (39.5%) resumed menstruation with a median time of 4 mo. Of about 14 patients who desired to conceive, four were pregnant and delivered good outcomes. Only one case was aborted. Therefore, the successful pregnancy rate was 28.6.
CONCLUSION
The oncology and reproductive outcomes of MOGCT treated by FSS are excellent. Many patients show a long survival time with normal menstruation. However, the obstetric outcome is not quite satisfactory.
PubMed: 36337312
DOI: 10.5306/wjco.v13.i10.802 -
Gynecologic Oncology Reports Oct 2022A pure ovarian dysgerminoma in a postmenopausal female is a rare phenomenon.
BACKGROUND
A pure ovarian dysgerminoma in a postmenopausal female is a rare phenomenon.
CASE
A 65-year-old female presented with a large pelvic mass. Following surgical debulking, the patient was diagnosed with FIGO Stage IIB ovarian dysgerminoma. She was treated with three cycles of etoposide and cisplatin and has been disease-free for 12 months.
CONCLUSION
Dysgerminomas in postmenopausal females are uncommon. Gynecologic oncologists should be familiar with the pathological diagnosis and treatment recommendations to achieve optimal outcomes.
PubMed: 36212092
DOI: 10.1016/j.gore.2022.101068 -
Indian Journal of Surgical Oncology Sep 2022Germ cell tumors (GCT) are an intriguing group of neoplasm having myriad clinical and morphological presentation. More and more transcription factors are being evaluated...
Germ cell tumors (GCT) are an intriguing group of neoplasm having myriad clinical and morphological presentation. More and more transcription factors are being evaluated for identification of same. To study the spectrum of GCTs in a tertiary care center and the use of a stem cell marker OCT4 as a diagnostic adjunct, a retrospective 5-year (2008-2013) study was carried out. Immunohistochemistry (IHC) with OCT4 was performed on all cases and IHC for α feto protein (AFP), CD30, and epithelial membrane antigen (EMA) as per requirement. Cohort included 73 cases (23 males and 50 females). Testicular and ovarian GCTs accounted for 95.83% and 35.71% respectively. In males, seminoma was the commonest (34.78%) followed by mixed GCT (26%). 17.85% of ovarian GCTs were malignant mostly constituted by dysgerminoma (18%). Benign mature cystic teratoma (MCT) constituted 50% of ovarian GCTs. OCT4 immunoexpression was seen in all cases of seminoma/dysgerminoma, embryonal carcinoma, immature teratoma, and seminomatous/embryomatous component of mixed GCTs. Pure yolk sac tumor (YST) and MCT were consistently negative. OCT4 was especially helpful in identification of mixed GCT. A panel of immunohistochemical markers would be a more ideal way to identify and clarify the components because correct identification of the components is important for therapeutic intervention and prognostication. OCT4 being a primordial germ cell marker predicts aggressive behavior and targeted therapy against this should be investigated.
PubMed: 36187544
DOI: 10.1007/s13193-022-01522-w -
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 -
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 -
Clinical Case Reports Jul 2022Swyer syndrome is a 46, XY karyotype, with pure gonadal dysgenesis and primary amenorrhea. These females have primordial Mullerian structures and seek medical attention...
Swyer syndrome is a 46, XY karyotype, with pure gonadal dysgenesis and primary amenorrhea. These females have primordial Mullerian structures and seek medical attention as they experience primary amenorrhea. Here, we report a 15-year-old girl, diagnosed as Swyer syndrome associated with left ovarian dysgerminoma.
PubMed: 35846908
DOI: 10.1002/ccr3.6083 -
Ginekologia Polska 2022Pure gonadal dysgenesis is a situation when the karyotype is 46, XY, but for various reasons there is a disorder of differentiation of Wolffian and Mullerian structures...
Pure gonadal dysgenesis is a situation when the karyotype is 46, XY, but for various reasons there is a disorder of differentiation of Wolffian and Mullerian structures and in consequence the phenotype is female. It is known that abdominal gonads and the presence of Y chromosome allow to qualify this condition as a high risk of tumor. In most cases breast development is limited because of lack or low level of estrogen. A 27-year-old patient with differences of sexual development (DSD), was admitted to the Department of Endocrinological Gynecology for a control examination. In the history: dysgerminoma, primary amenorrhea and ambiguous karyotype. The patient has not taken hormonal replacement therapy. The breast development is Tanner stage V.
Topics: Dysgerminoma; Female; Gonadal Dysgenesis; Gonadal Dysgenesis, 46,XY; Gonads; Humans; Ovarian Neoplasms
PubMed: 35730347
DOI: 10.5603/GP.a2022.0029