Molecular Genetic Analysis of Bilateral Ovarian Germ Cell Tumors

Hennes, Emily R; Zahn, Susanne; Lopes, Luiz Fernando; Schönberger, Stefan; Leuschner, Ivo; Göbel, U.; Calaminus, Gabriele; Schneider, Dominik T.

doi:10.1055/s-0032-1327606

Cited by 18 publications

(17 citation statements)

References 14 publications

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“…Chromosomal studies were not available in that report. An interesting finding was reported by Hennes et al . in their review of the Kiel German Childhood Tumor Registry.…”

Section: Discussionmentioning

confidence: 55%

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Gonadal dysgenesis is associated with worse outcomes in patients with ovarian nondysgerminomatous tumors: A report of the Children's Oncology Group AGCT 0132 study

Dicken

Billmire

Krailo

et al. 2017

Pediatric Blood & Cancer

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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. Patients were compared with respect to event-free survival (EFS) and overall survival (OS). Results: Nine patients with GD, including seven with gonadoblastoma (mean age, 9.3 years), were compared to 100 non-GD patients (mean age, 12.1 years). The estimated 3-year EFS for patients with GD was 66.7% (95% CI 28.2–87.8%) and for non-GD patients was 88.8% (95% CI 80.2–93.8%). The estimated 3-year OS for patients with GD was 87.5% (95% CI 38.7–98.1%) and for non-GD patients was 97.6% (95% CI of 90.6–99.4%). Conclusion: Patients presenting with nongerminomatous malignant ovarian GCTs in the context of GD have a higher rate of events and death than counterparts with normal gonads. These findings emphasize the importance of noting a contralateral streak ovary or gonadoblastoma at histology for any ovarian GCT and support the recommendation for early bilateral gonadectomy in patients known to have GD with Y chromosome material. In contrast to those with pure dysgerminoma, these patients may represent a high-risk group that requires a more aggressive chemotherapy regimen.

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“…Chromosomal studies were not available in that report. An interesting finding was reported by Hennes et al . in their review of the Kiel German Childhood Tumor Registry.…”

Section: Discussionmentioning

confidence: 55%

“…Chromosomal studies were not available in that report. An interesting finding was reported by Hennes et al19 in their review of the Kiel German Childhood Tumor Registry. They chose to undertake molecular genetic analysis of their ovarian GCT patients with bilateral tumors at diagnosis.…”

mentioning

confidence: 55%

Gonadal dysgenesis is associated with worse outcomes in patients with ovarian nondysgerminomatous tumors: A report of the Children's Oncology Group AGCT 0132 study

Dicken

Billmire

Krailo

et al. 2017

Pediatric Blood & Cancer

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show abstract

“…In addition, patients should also be screened for thyroid disease during follow-up, in particular in case of positive family history for multinodular goitre. While in ovarian germ cell tumours, kit mutations or sex chromosomal aberrations are associated with bilateral disease [6,7], the development of bilateral OSCSTs and secondary tumours can be explained in the context of constitutional DICER1 mutation. Thus, genetic counselling and testing of DICER1 should be recommended in SLCTs, because a positive finding might have implications for follow-up.…”

Section: Discussionmentioning

confidence: 99%

Ovarian Sertoli Leydig cell tumours in children and adolescents: An analysis of the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT)

Schneider¹,

Orbach²,

Cecchetto³

et al. 2015

European Journal of Cancer

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“…To summarise, a woman's reproductive history does not seem to influence her risk of GCTs. Gonadal dysgenesis is associated with risk of GCTs, 28,29 but not SCSTs. This suggests that development of GCTs is more genetically controlled, given that gonadal dysgenesis is secondary to chromosomal anomalies or mutations in genes related to the urogenital ridge or sex differentiation.…”

Section: Germ Cell Tumoursmentioning

confidence: 99%

“…We included women recorded with a pregnancy lasting longer than 22 weeks in the MBRs who later developed malignant SCSTs and GCTs and were registered in a cancer registry between 1973 and 2011 in Denmark, 1987and 2012in Finland, 1967 and 2013 in Norway and 1973 and 2013 in Sweden (Table 1). Cases were free of other cancers at time of ovarian cancer diagnosis Ovarian cancer was defined by ICD-10/ICD-O-3 code C56.9, and by ICD-7 code 175.0 in Sweden before 1993, and ICD-O/2/ICD-O/3 codes and WHO/HS/CAN/C24.1 codes, as defined by the International Agency for Research on Cancer, 23 were used to identify SCSTs and GCTs (supplementary Table 1).…”

mentioning

confidence: 99%

Pregnancy-related risk factors for sex cord-stromal tumours and germ cell tumours in parous women: a registry-based study

et al. 2020

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BACKGROUND: Non-epithelial ovarian cancers are divided into sex cord-stromal tumours (SCSTs) and germ cell tumours (GCTs). Whereas parity and other pregnancy-related factors are protective for epithelial ovarian cancer, their associations with SCSTs and GCTs remains unclear. METHODS: Using data from the medical birth registries from Denmark, Finland, Norway and Sweden, we compared all parous women with a diagnosis of SCSTs (n = 420) or GCTs (n = 345) 1970-2013 with up to 10 parous controls (SCSTs n = 4041; GCTs n = 2942) matched on the cases' birth year and country. We used conditional logistic regression to estimate odds ratios (ORs) with 95% confidence intervals (CIs) of associations between pregnancy-related factors and SCSTs and GCTs. RESULTS: The risk of SCSTs, but not GCTs, decreased with higher age at last birth [≥40 versus <25 years: OR 0.48 (95% CI 0.23-0.98)]. The risk of SCSTs (but not GCTs) also decreased with shorter time since last birth. Number of births, preterm birth, preeclampsia, and offspring size were not associated with risk of SCSTs or GCTs. CONCLUSIONS: We found a decreased risk of SCSTs with higher age at last birth and shorter time since last birth. The risk of SCSTs (but not GCTs) may be influenced by the woman's reproductive history.

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Molecular Genetic Analysis of Bilateral Ovarian Germ Cell Tumors

Cited by 18 publications

References 14 publications

Gonadal dysgenesis is associated with worse outcomes in patients with ovarian nondysgerminomatous tumors: A report of the Children's Oncology Group AGCT 0132 study

Gonadal dysgenesis is associated with worse outcomes in patients with ovarian nondysgerminomatous tumors: A report of the Children's Oncology Group AGCT 0132 study

Ovarian Sertoli Leydig cell tumours in children and adolescents: An analysis of the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT)

Pregnancy-related risk factors for sex cord-stromal tumours and germ cell tumours in parous women: a registry-based study

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