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Dysgerminoma

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https://www.readbyqxmd.com/read/29331749/paediatric-dysgerminoma-results-of-three-consecutive-french-germ-cell-tumours-clinical-studies-tgm-85-90-95-with-late-effects-study
#1
Gwénaëlle Duhil de Bénazé, Hélène Pacquement, Cécile Faure-Conter, Catherine Patte, Daniel Orbach, Nadège Corradini, Claire Berger, Hélène Sudour-Bonnange, Cécile Vérité, Hélène Martelli, Brice Fresneau
METHODS: French patients (≤18years) treated for dysgerminoma between 1985 and 2005 in TGM-85, 90, 95 protocols were included. Treatment was based on primary unilateral oophorectomy followed by prophylactic lymph node irradiation (1985-1998) or a wait-and-see strategy (1998-2005) for localised completely resected tumours (pS1) or by platinum-based chemotherapy for advanced diseases. RESULTS: Forty-eight patients (median age 12.8 years) were included. Six patients had gonadal dysgenesis...
January 10, 2018: European Journal of Cancer
https://www.readbyqxmd.com/read/29317190/normal-pelvic-ultrasound-or-mri-does-not-rule-out-neoplasm-in-patients-with-gonadal-dysgenesis-and-y-chromosome-material
#2
Kristin M Ebert, Geri D Hewitt, Justin A Indyk, Katherine A McCracken, Leena Nahata, Venkata R Jayanthi
INTRODUCTION: Patients with gonadal dysgenesis (GD) with a Y chromosome have an increased risk of gonadal neoplasm. Few data exist on the ability of imaging to detect malignancy in intra-abdominal gonads in these patients. OBJECTIVE: We aimed to determine the correlation between preoperative imaging findings and gonadal pathology in GD patients with Y chromosome material. METHODS: A retrospective review was performed of patients with XY or XO/XY GD who underwent gonadectomy at our institution from 2003 to 2017...
December 23, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29290327/-the-value-of-immunohistochemistry-using-smarca4-brg1-in-the-diagnosis-of-small-cell-ovarian-carcinoma-hypercalcemic-type-a-report-of-two-cases
#3
Gema Ruiz-García, M Amparo Torroba-Carón, Belén Ferri-Ñíguez, Miriam Lencina-Guardiola, Francisco García-Molina, Enrique Martínez-Barba
Small cell carcinoma of ovary-hypercalcemic type is an undifferentiated carcinoma. We describe two cases in women aged 32 and 29. Both presented with large masses and complete surgical extirpation was impossible. Histologically, the images were similar, with diffuse cell proliferation, accompanied by the presence of follicle-like spaces. In both cases it was necessary to make a differential diagnosis with entities such as adult or juvenile granulosa cell tumour, small cell carcinoma of pulmonary type, dysgerminoma and even peripheral neuroectodermal tumour...
January 2018: Revista Española de Patología
https://www.readbyqxmd.com/read/29286555/gonadal-dysgenesis-is-associated-with-worse-outcomes-in-patients-with-ovarian-nondysgerminomatous-tumors-a-report-of-the-children-s-oncology-group-agct-0132-study
#4
Bryan J Dicken, Deborah F Billmire, Mark Krailo, Caihong Xia, Furqan Shaikh, John W Cullen, Thomas A Olson, Farzana Pashankar, Marcio H Malogolowkin, James F Amatruda, Frederick J Rescorla, Rachel A Egler, Jonathan H Ross, Carlos Rodriguez-Galindo, A Lindsay Frazier
PURPOSE: In this report, we characterize the timing and behavior of malignant ovarian germ cell tumors (GCTs) in pediatric patients with dysgenetic gonads compared to those with normal gonadal development. PATIENTS AND METHODS: Patients from the Children's Oncology Group AGCT0132 with malignant ovarian GCTs were included. Within this population, we sought to identify patients with gonadoblastoma, streak ovaries, or other evidence of gonadal dysgenesis (GD). Patients with malignant GCTs containing one or more of the following histologies-yolk sac tumor, embryonal carcinoma, or choriocarcinoma-were included...
December 29, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29248931/molecular-detection-and-incidence-of-y-chromosomal-material-in-patients-with-turner-syndrome
#5
Aleksandra Rojek, Monika Obara-Moszynska, Zofia Kolesinska, Barbara Rabska-Pietrzak, Marek Niedziela
The presence of a Y chromosome in patients with Turner syndrome (TS) is a risk factor for the development of gonadal tumor and/or virilization. With conventional cytogenetic analysis, some cells containing a Y chromosome can be missed. The aim of this study was to determine the presence and incidence of Y chromosome-derived material in TS patients using PCR and the markers SRY, DYZ1, DYZ3, DYS132, ZFY, and TSPY. Fifty-five TS patients (aged 5.5-26.75 years) were analyzed. A total of 17/55 (30.9%) were Y-positive, but only 7/17 had a Y chromosome in their karyotype and underwent gonadectomy...
December 16, 2017: Sexual Development: Genetics, Molecular Biology, Evolution, Endocrinology, Embryology, and Pathology of Sex Determination and Differentiation
https://www.readbyqxmd.com/read/29187016/adolescent-female-with-turner-s-syndrome-and-46-x-der-y-del-y-p11-2-del-q11-2-karyotype-with-gonadoblastoma-and-dysgerminoma
#6
Barenya Mukerji, Eli Balshan, Robert Haderer, Wendy Shertz, Robert Graebe
Gonadal dysgenesis patients with Y chromosomal material are subject to increased risk for germ cell tumors. We report a case of an adolescent female presenting with Turner-like syndrome with primary amenorrhea and Tanner stage 1 breast development. Karyotype showed one X chromosome and a minute pericentromeric fragment of Y chromosome without any functional Y genes in all the cells, unlike a mosaic pattern, represented as 46,X,der(Y)del(Y)(p11.2)del(q11.2). Laparoscopic bilateral gonadectomy was performed due to presence of Y chromosome material and histopathology confirmed gonadoblastoma with a focus of dysgerminoma of the right ovary...
November 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29073306/-y-chromosome-in-turner-syndrome
#7
REVIEW
Aleksandra Rojek, Karolina Kwasiuk, Monika Obara-Moszyńska, Zofia Kolesińska, Marek Niedziela
Turner syndrome (TS) is an inherited genetic disorder caused by numerical and/or structural chromosome X aberrations occurring at a frequency of 1:1200-1:2500 live-born girls. The most common karyotype is X chromosome monosomy (45,X) (approximately 50-60% of cases). Approximately 5-6% of patients may have abnormal Y chromosome or mosaicism characterized by the coexistence of 45,X cell line with cell line in which all or part of chromosome Y is present. In patients with TS who have all or fragmented genetic material from chromosome Y there is a substantial risk of cancerous lesions in these dysgenetic gonads...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
https://www.readbyqxmd.com/read/29069951/familial-swyer-syndrome-a-rare-genetic-entity
#8
Manilal Banoth, Ramana Reddy Naru, Mohammed Basheeruddin Inamdar, Amit Kumar Chowhan
Swyer syndrome is a pure gonadal dysgenesis associated with a 46 XY karyotype and primary amenorrhea in a phenotypic female. Individuals in this syndrome are at an increased risk for development of gonadal malignancies. Swyer syndrome (gonadal dysgenesis) running in families is rare event and few such scenarios were reported in the literature. Here we are presenting this rare entity involving three affected siblings born to a non-consanguineous couple. Index case - A 23-year-old female with primary amenorrhea is presented with a mass per abdomen...
October 26, 2017: Gynecological Endocrinology
https://www.readbyqxmd.com/read/28969219/giving-birth-after-fertility-sparing-treatment-for-a-yolk-sac-tumour-case-report
#9
Georgios-Marios Makris, Alexandros Fotiou, Georgios Chrelias, Marco-Johannes Battista, Charalampos Chrelias
Yolk Sac Tumours (YSTs) of the ovary, also called Endodermal Sinus Tumours (ESTs), are the second most common Malignant Ovarian Germ Cell Tumours (MOGCTs), after dysgerminomas. YSTs occur primarily in children and young women. We present a case report of a 20-year-old woman who had been diagnosed with YST (tumour diameter of 29 cm). The patient underwent fertility sparing surgery and was subsequently treated with adjuvant chemotherapy (four cycles of bleomycin, etoposide and cisplatin). Two years after the diagnosis of YST, she successfully gave birth to a healthy girl (birth weight 3,500 g, Apgar score of 9-10 and a spontaneous vaginal delivery)...
August 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28904580/extradural-spinal-metastasis-in-ovarian-mixed-germ-cell-tumor
#10
Deepashu Sachdeva, Vikas Kumar, Ravindra Kumar Saran, Arvind Kumar Srivastava
Ovarian germ cell tumors (GCTs) are rare and affect mainly young girls and women. Two histological groups are distinguished: dysgerminomas and nondysgerminomatous tumors. These tumors have initial good responses to surgery and chemotherapy in 80% cases, but >75% of patients die due to complications of disease progression. There are very few case reports of mixed GCT with extradural spine metastases. We report a rare case of a 17-year-old girl who had undergone left salpingo-oophorectomy with omental and peritoneal biopsy for ovarian GCT with extradural spinal metastasis...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28800577/case-report-use-of-tumor-and-germline-y-chromosomal-analysis-to-guide-surgical-management-in-a-46-xx-female-presenting-with-gonadoblastoma-with-dysgerminoma
#11
Jeanna M McCuaig, Abdul Noor, Barry Rosen, Robert F Casper, Frederic Mitri, Terence Colgan, Raymond H Kim
Gonadoblastomas are rare mixed gonadal tumors that are almost always found in individuals with 46, XY karyotype or some other form of Y chromosome mosaicism. It is extremely rare to diagnose gonadoblastoma in phenotypically normal 46, XX females. Herein, we present a 20-year-old 46, XX female diagnosed with gonadoblastoma and dysgerminoma. Use of cytogenetic and molecular analyses to identify the presence of Y chromosome material in peripheral blood, gonadal, and tumor tissue can exclude mosaicism to provide reassurance to undertake conservative surgical management and preserve fertility...
September 2017: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/28750644/a-retrospective-study-of-reproductive-outcomes-after-fertility-sparing-surgery-and-postoperative-adjuvant-chemotherapy-in-malignant-ovarian-germ-cell-tumors-and-sex-cord-stromal-tumors
#12
Ning Zhang, Ruifang Chen, Keqin Hua, Ying Zhang
BACKGROUND: To retrospectively investigate reproductive outcomes after fertility-sparing surgery and postoperative adjuvant chemotherapy in malignant ovarian germ cell tumors (MOGCT) and sex cord-stromal tumors (SCST). METHODS: Data from 32 MOGCT (6 dysgerminomas, 6 yolk sac tumors, 17 immature teratomas, and 3 mixed germ cell tumors) and 9 SCST (4 granulosa cell tumors and 5 sertoli-leydig cell tumors) aged from 18 to 35, treated in the Obstetrics and Gynecology Hospital of Fudan University from October 2003 to October 2013 were collected and analyzed...
July 27, 2017: Journal of Ovarian Research
https://www.readbyqxmd.com/read/28585395/epithelial-mesenchymal-transition-markers-in-malignant-ovarian-germ-cell-tumors
#13
Olesya Solheim, Mette Førsund, Claes G Tropé, Sigrid Marie Kraggerud, Jahn M Nesland, Ben Davidson
The purpose of this study was to determine the expression and potential clinical role of epithelial-to-mesenchymal transition (EMT)-related factors in malignant ovarian germ cell tumors (MOGCT). Protein expression of E-cadherin, N-cadherin, P-cadherin, Zeb1, HMGA2, and vimentin by immunohistochemistry was analyzed in 42 MOGCT from patients treated in Norway during the period 1981-2001. Expression was analyzed for association with clinicopathologic parameters. E-cadherin (p = 0.016) and HMGA2 (p = 0.002) expression was significantly higher in immature teratomas and yolk sac tumors compared with dysgerminomas...
September 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/28520571/is-omentectomy-mandatory-among-early-stage-i-ii-malignant-ovarian-germ-cell-tumor-patients-a-retrospective-study-of-223-cases
#14
Wenyan Xu, Yanfang Li
OBJECTIVE: The aim of the study was to investigate whether omentectomy (OMT) is necessary in the operation for apparently early stage malignant ovarian germ cell tumors (MOGCTs). METHODS AND MATERIALS: Searching medical records database of Sun Yat-sen University Cancer Center from January 1, 1966, to November 30, 2015, patients with MOGCTs were identified and their age, year of diagnosis, tumor grade, histologic subtype, International Federation of Gynecology and Obstetrics stage, nodal findings, gross observation of omentum, and performance of OMT were assessed...
September 2017: International Journal of Gynecological Cancer
https://www.readbyqxmd.com/read/28469317/impact-of-surepath-%C3%A2-liquid-based-preparation-in-cytological-analysis-of-peritoneal-washing-in-practice-of-gynecologic-oncology
#15
Ruchita Tyagi, Nalini Gupta, Priyanka Bhagat, Shalini Gainder, Bhavna Rai, L K Dhaliwal, Arvind Rajwanshi
CONTEXT: Peritoneal washing is performed for staging of gynecologic tumors to detect subclinical intraperitoneal metastases. AIM: The aim of the present study was to assess the impact of SurePath™ liquid-based cytology (LBC) in peritoneal washing in various gynecological malignancies. SETTINGS AND DESIGN: An audit of peritoneal-fluid/washing (January 2012 to July 2013) was performed with corresponding gynecologic specimens. All peritoneal washings were processed using both conventional and LBC technique...
April 2017: Journal of Cytology
https://www.readbyqxmd.com/read/28449306/malignant-ovarian-germ-cell-tumors-in-pediatric-patients-the-aieop-associazione-italiana-ematologia-oncologia-pediatrica-study
#16
M Terenziani, G Bisogno, R Boldrini, G Cecchetto, M Conte, L Boschetti, M D De Pasquale, D Biasoni, A Inserra, F Siracusa, M E Basso, F De Leonardis, D Di Pinto, F Barretta, F Spreafico, P D'Angelo
OBJECTIVE: Malignant ovarian germ cell tumors (MOGCT) carry an excellent prognosis, and the treatment aims to achieve results with the least possible treatment-related morbidity. The aim of this study was to assess the outcomes of pediatric patients with MOGCT. METHODS: Patients were treated according to their stage: surgery and surveillance for stage I; a modified bleomycin-etoposide-cisplatin (BEP) regimen for stages II (three cycles), III, and IV (three cycles) with surgery on residual disease...
November 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28434637/clinical-heterogeneity-in-children-with-gonadal-dysgenesis-associated-with-non-mosaic-46-xy-karyotype
#17
Y S Wong, Y H Tam, K K Y Pang, K F To, S S C Chan, K W Chan, K H Lee
INTRODUCTION: Gonadal dysgenesis is unique in disorders of sex development (DSD), in that it can be associated with 46,XX, 46,XY or mosaic 45,X/46,XY karyotypes. Gonadal dysgenesis can be partial or complete. Gonadal dysgenesis associated with the Y-chromosome has increased risk of gonadal germ cell neoplasms. Most of the literature focus on 45,X/46,XY gonadal dysgenesis, while there are scanty data on the condition when the karyotype is non-mosaic 46,XY. OBJECTIVE: To investigate the diversity of clinical pictures of children presenting with 46,XY DSD due to gonadal dysgenesis...
October 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28166073/ovarian-tumors-pattern-of-histomorphological-types-a-10-years-study-in-a-tertiary-referral-center-and-review-of-literature
#18
REVIEW
A Ghosh, D Ghartimagar, S Thapa, B Sathian, R Narasimhan, O P Talwar
Background Ovarian cancer accounts for 6% of all cancers in females. Among cancers of female genital tract, the incidence of ovarian cancers ranks below only carcinoma of the cervix and the endometrium. Objective To find the frequency of different types of histomorphological types and their association with age, side and size distribution. Method This study is a hospital based retrospective study carried out in the Department of Pathology, Manipal Teaching Hospital over a time period from Jan. 2006 to Sep. 2015...
April 2016: Kathmandu University Medical Journal (KUMJ)
https://www.readbyqxmd.com/read/28137901/complications-of-dysgerminoma-meeting-the-health-needs-of-patients-in-conflict-zones
#19
Lili Hayari, Erez Shir On, Andrei Fedorenko, Szvalb Sergio, Jamal Zidan, Evgeny Solomonov
A symptom of prolonged conflict is the destruction of infrastructure and healthcare systems. While the need for acute trauma services is obvious in conflict zones, patients with chronic diseases also require care. This report describes the clinical course of a young teenage girl with a large mid pelvic tumour originating from the left ovary and reaching the umbilicus. She presented with acute abdominal pain and underwent surgery in a healthcare facility within a conflict zone. She was then transferred to a neighbouring country for continuing care...
January 30, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28030592/a-novel-missense-mutation-224g-t-r75m-in-sry-coding-region-interferes-with-nuclear-import-and-results-in-46-xy-complete-gonadal-dysgenesis
#20
Wufang Fan, Bei Wang, Shanshan He, Tengfei Zhang, Chenxing Yin, Yunping Chen, Shuqi Zheng, Jixia Zhang, Lin Li
SRY-mutation-caused sex reversal is a rare disease and mostly associated with a de novo mutation since the patients with defective SRY is infertile. There are many reports about SRY-mutation associated 46, XY ovarian disorder of sex development (DSD), but few described their molecular mechanism. Here we report a de novo mutation 224G>T (R75M) in SRY associated with a phenotypic female, 46, XY karyotype and dysgerminoma. The wild and mutated SRY were cloned into recombinant plasmid and expressed in cells in vitro, the result showed the mutated SRY is greatly accumulated in cytoplasm while the wild type SRY is mostly localized in nucleus...
2016: PloS One
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