-
Gynecologic Oncology Reports Jun 2023While Non-Hodgkin Lymphoma (NHL) often involves the ovaries at time of autopsy, it is rarely present at the time of diagnosis. Here we present a case of a 20-year-old...
While Non-Hodgkin Lymphoma (NHL) often involves the ovaries at time of autopsy, it is rarely present at the time of diagnosis. Here we present a case of a 20-year-old who presented with a large adnexal mass and elevated B-HCG, CA-125, and LDH. The patient underwent exploratory laparotomy, and frozen section of the left ovarian mass was suspected to be a dysgerminoma. Final pathologic diagnosis was Ann Arbor stage IVE Diffuse Large B-Cell Lymphoma, germinal center subtype. Patient is currently undergoing chemotherapy and has received the 3 of a planned 6 cycles of R-CHOP.
PubMed: 37293350
DOI: 10.1016/j.gore.2023.101212 -
Cureus Apr 2023The 46,XY disorder of sexual development (DSD) is a rare congenital condition characterized by a 46,XY karyotype associated with complete or disturbed female gonadal...
The 46,XY disorder of sexual development (DSD) is a rare congenital condition characterized by a 46,XY karyotype associated with complete or disturbed female gonadal development and a non-virilized phenotype. The presence of Y chromosome material in these patients' karyotypes increases the risk of germ cell tumor development. The present study reports a unique case of a 16-year-old phenotypically female patient presenting with primary amenorrhea, who was later diagnosed with 46,XY DSD. After bilateral salpingo-oophorectomy, the patient was diagnosed with stage IIIC dysgerminoma. The patient received four cycles of chemotherapy and showed a good response. The patient is currently alive and well, with no evidence of disease after the residual lymph node resection.
PubMed: 37252506
DOI: 10.7759/cureus.38149 -
Journal of the ASEAN Federation of... 2023Complete gonadal dysgenesis with 46,XY karyotype is a clinical condition characterized by the absence of testicular tissue but with the presence of typical Müllerian...
Complete gonadal dysgenesis with 46,XY karyotype is a clinical condition characterized by the absence of testicular tissue but with the presence of typical Müllerian structures in a phenotypically female individual. The condition presents as primary amenorrhoea or delayed puberty. Eventually, malignant neoplasms may arise. We report a case of a 16-year-old Indian male with Swyer syndrome presenting with primary amenorrhoea and with an earlier diagnosis of a malignant dysgerminoma in the right ovary.
Topics: Male; Female; Humans; Adolescent; Dysgerminoma; Ovarian Neoplasms; Amenorrhea; Gonadal Dysgenesis, 46,XY
PubMed: 37252405
DOI: 10.15605/jafes.038.01.15 -
European Journal of Obstetrics,... Jul 2023Rare tumour management is challenging for clinicians as evidence bases are limited and clinical trials are difficult to conduct. It is even more difficult for patients...
OBJECTIVE
Rare tumour management is challenging for clinicians as evidence bases are limited and clinical trials are difficult to conduct. It is even more difficult for patients where self-reliance alone is insufficient to overcome the challenges of navigating care which is often poorly evidence based. In Ireland, a national Gestational Trophoblastic Disease (GTD) service was established as one of 3 initiatives for rare tumours by the National Cancer Control Programme. The service has a national clinical lead, a dedicated supportive nursing service and a clinical biochemistry liaison team. This study sought to assess the impact of a GTD centre using national clinical guidelines and integrating and networking with European and International GTD groups on the clinical management of challenging GTD cases and to consider the application of this model of care to other rare tumour management.
STUDY DESIGN
In this article, we analyse the impact of a national GTD service on five challenging cases, and review how the service affects patient management in this rare tumour type. These cases were selected from a cohort of patients who were voluntarily registered in the service based on the diagnostic management dilemma they posed.
RESULTS
Case management was impacted by the identification of GTD mimics, the provision of lifesaving treatment of metastatic choriocarcinoma with brain metastasis, networking with international colleagues, the identification of early relapse, the use of genetics to differentiate treatment pathways and prognosis, and supportive supervision of treatment courses of up to 2 years of therapy in a cohort of patients starting or completing families.
CONCLUSION
The National GTD service could be an exemplar for the management of rare tumours (such as cholangiocarcinoma) in our jurisdiction which could benefit from a similar constellation of supports. Our study demonstrates the importance of a nominated national clinical lead, dedicated nurse navigator support, registration of cases and networking. The impact of our service would be greater if registration was mandatory rather than voluntary. Such a measure would also ensure equity of access for patients to the service, assist in quantifying the need for resourcing and facilitate research to improve outcomes.
Topics: Pregnancy; Female; Humans; Gestational Trophoblastic Disease; Prognosis; Ireland; Neoplasms, Second Primary; Uterine Neoplasms
PubMed: 37224702
DOI: 10.1016/j.ejogrb.2023.05.016 -
International Journal of Gynecological... Nov 2023The objective of this study was to analyze the expression and potential clinical role of cancer stem cell (CSC) markers in malignant ovarian germ cell tumors (MOGCT)....
The objective of this study was to analyze the expression and potential clinical role of cancer stem cell (CSC) markers in malignant ovarian germ cell tumors (MOGCT). CD34, CD44, and SOX2 protein expression by immunohistochemistry was analyzed in 49 MOGCT from patients treated in Norway during the period 1980-2011. Expression was analyzed for association with tumor type and clinicopathologic parameters. Tumors were diagnosed as dysgerminoma (DG; n=15), immature teratoma (IT; n=15), yolk sac tumor (YST; n=12), embryonal carcinoma (n=2), and mixed MOGCT (n=5). Tumor cell CD34 expression was significantly more common in YST, whereas stromal expression was only seen in IT (both P <0.001). CD44 was infrequently expressed, most often focally, in tumor cells, particularly in YST ( P =0.026). CD44 was widely expressed in leukocytes, most prominently in DG. SOX2 was most frequently expressed in IT, with predominantly focal expression in some YST and uniform absence in DG ( P <0.001). Stromal CD34 ( P =0.012) and tumor cell SOX2 expression ( P =0.004) were negatively associated with the involvement of the ovarian surface, presumably due to the low incidence of this event in IT. No significant association was found between CSC marker expression and other clinicopathologic parameters, including age, laterality, tumor diameter, and FIGO stage. In conclusion, CSC markers are differentially expressed in various MOGCT types, suggesting differences in the regulation of cancer-related processes. Expression of CD34, CD44, and SOX2 does not appear to be associated with clinical parameters in this patient group.
PubMed: 37186896
DOI: 10.1097/PGP.0000000000000947 -
Diagnostics (Basel, Switzerland) Apr 2023An immature teratoma is a germinal malignant tumor composed of three germ cell layers, occurring more frequently in young women. It is the second most frequent among the... (Review)
Review
An immature teratoma is a germinal malignant tumor composed of three germ cell layers, occurring more frequently in young women. It is the second most frequent among the malignant germinal tumors after dysgerminoma, and it is the only neoplasm with germ cells that are histologically graded. Even if we do not have a consensus regarding its therapeutical management, it has a good prognosis, with an excellent overall survival rate and good fertility preservation. More studies are needed regarding the necessity of adjuvant chemotherapy in pediatric oncology, and because of chemotherapy's long-term adverse effects, surveillance or a targeted treatment is preferred, but the main therapy is fertility-sparing surgery. Special attention should be given to the genetic mapping of the histological pieces for patient risk stratification due to its value in prognosis and future treatment.
PubMed: 37174909
DOI: 10.3390/diagnostics13091516 -
International Journal of Surgical... Feb 2024Malignant ovarian germ cell tumors represent small percentage of malignant ovarian neoplasms but they affect significantly young age group.
BACKGROUND
Malignant ovarian germ cell tumors represent small percentage of malignant ovarian neoplasms but they affect significantly young age group.
AIM OF THE STUDY
To investigate the immunohistochemical expression of p16 tumor suppressor protein in malignant ovarian germ cell tumors.
MATERIALS AND METHODS
Twenty-two malignant ovarian germ cell tumors (five dysgerminoma, eight immature teratoma, and nine yolk sac tumors), twenty mature cystic teratoma tumors and twenty normal ovarian tissue were immunohistochemically stained with p16 monoclonal antibody. Ki67 immunohistochemical staining was done for malignant ovarian germ cell tumors to assess proliferation.
RESULTS
We found that p16 tumor suppressor protein is overexpressed in all malignant ovarian germ cell tumors in both nuclear and cytoplasmic locations compared to control and to mature cystic teratoma (-value <0.001). Cytoplasmic p16 expression was significantly correlated to Ki67 proliferation index in malignant ovarian germ cell tumors (-value = 0.033, = 0.445).
CONCLUSION
Overexpression of p16 in malignant ovarian germ cell tumors denotes that dysfunction of the cyclin dependent kinase pathway is involved in tumorigenesis of malignant ovarian germ cell tumors.
Topics: Female; Humans; Ki-67 Antigen; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Teratoma; Tumor Suppressor Proteins
PubMed: 37170541
DOI: 10.1177/10668969231168358 -
Radiologic Clinics of North America Jul 2023Ovarian malignant germ cell tumors are a diverse set of masses originating from the primitive gonadal germ cells, often in young females. They have useful imaging and... (Review)
Review
Ovarian malignant germ cell tumors are a diverse set of masses originating from the primitive gonadal germ cells, often in young females. They have useful imaging and clinical features, including serum tumor marker elevation, that may aid the radiologist at the time of diagnosis, and also during follow-up. Accurate and timely diagnosis is essential, as standard-of-care therapies lead to a high rate of cancer remission.
Topics: Female; Humans; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Biomarkers, Tumor
PubMed: 37169425
DOI: 10.1016/j.rcl.2023.02.004 -
The Turkish Journal of Pediatrics 2023Primary ovarian tumors are rare in the pediatric age group. We reviewed our 40-year experience with ovarian tumors to evalute the clinical features and treatment results...
BACKGROUND
Primary ovarian tumors are rare in the pediatric age group. We reviewed our 40-year experience with ovarian tumors to evalute the clinical features and treatment results in a single institution.
METHODS
Between January 1975 and October 2015, 124 girls with primary ovarian tumor were diagnosed and treated in our center. Tumors were identified with biopsy or total resection and/or serum markers. Seventy four children were included in the treatment analysis.
RESULTS
Median age for 124 children was 11.0 years (0.73-17.63). The main complaint was abdominal pain in 85 patients (68.5%). One hundred and five patients (84.6%) had total one-sided salpingo-oophorectomy and five patients had bilateral salpingo-oophorectomy. Amongst 124 cases, 29 patients had mature teratoma, which was the most common tumor in this study. Dysgerminoma (n=21) was the most common malignant histopathologic type. Stage I disease was diagnosed in 57.2% and stage IV in 6.6% of the patients. Five year overall survival (OS) and event-free survival (EFS) for 124 children were 82.5% and 76.3% respectively. For 74 children who received treatment, 5-year OS and EFS were 75.2% and 67.1%, respectively. Age (p < 0.017), histopathological subgroup (p < 0.001), stage (p =0.003) and chemotherapy protocols (p =0.049) were significant prognostic factors for OS.
CONCLUSIONS
The survival rates in children with ovarian tumors were comparable with studies in the literature. Although patients treated with platin based regimens had better survival rates, prognosis was still poor for the patients in advanced stages. This should be the focus for further studies and improvements.
Topics: Child; Female; Humans; Antineoplastic Combined Chemotherapy Protocols; Neoplasm Staging; Ovarian Neoplasms; Prognosis; Retrospective Studies; Treatment Outcome
PubMed: 37114690
DOI: 10.24953/turkjped.2022.659 -
Diagnostic and Interventional Radiology... Jan 2023Ovarian dysgerminoma (OD) is a rare germ cell tumor accounting for 1%-2% of all malignant ovarian tumors and is generally associated with a good prognosis. The condition...
Ovarian dysgerminoma (OD) is a rare germ cell tumor accounting for 1%-2% of all malignant ovarian tumors and is generally associated with a good prognosis. The condition is more frequent in young women and can arise in dysgenetic gonads that contain gonadoblastomas. While the definitive diagnosis of OD is only possible histologically, certain radiological features can provide facilitating clues. A large, unilateral, solid, lobulated ovarian tumor with markedly enhancing septa should raise the suspicion of OD in young women. Serum lactate dehydrogenase is characteristically elevated in this tumor type and can complement its diagnosis and postoperative follow-up; however, it is a nonspecific marker. Moreover, knowing the mimickers of OD is essential to optimizing the radiological image interpretation and allowing for adequate management and timely treatment. Therefore, in this article, the radiological and clinical-pathologic features of ODs were reviewed to allow radiologists to become familiarized with them and narrow the diagnostic possibilities when facing this type of tumor.
Topics: Female; Humans; Dysgerminoma; Ovarian Neoplasms; Neoplasms, Germ Cell and Embryonal; Radiography
PubMed: 36959710
DOI: 10.5152/dir.2022.21317