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

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 Rescorla, Rachel A. Egler, Jonathan H. Ross, Carlos Rodriguez-Galindo, A. Lindsay Frazier

Research output: Contribution to journalArticle

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Abstract

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.

Original languageEnglish (US)
JournalPediatric Blood and Cancer
DOIs
StateAccepted/In press - Jan 1 2018

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Gonadal Dysgenesis
Neoplasms
Germ Cell and Embryonal Neoplasms
Gonadoblastoma
Gonads
Disease-Free Survival
Ovary
Histology
Dysgerminoma
Embryonal Carcinoma
Endodermal Sinus Tumor
Choriocarcinoma
Survival
Y Chromosome

Keywords

  • Gonadal dysgenesis
  • Malignant ovarian germ cell tumor
  • Pediatric outcome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

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, Bryan J.; Billmire, Deborah F.; Krailo, Mark; Xia, Caihong; Shaikh, Furqan; Cullen, John W.; Olson, Thomas A.; Pashankar, Farzana; Malogolowkin, Marcio H.; Amatruda, James F.; Rescorla, Frederick; Egler, Rachel A.; Ross, Jonathan H.; Rodriguez-Galindo, Carlos; Frazier, A. Lindsay.

In: Pediatric Blood and Cancer, 01.01.2018.

Research output: Contribution to journalArticle

Dicken, BJ, Billmire, DF, Krailo, M, Xia, C, Shaikh, F, Cullen, JW, Olson, TA, Pashankar, F, Malogolowkin, MH, Amatruda, JF, Rescorla, F, Egler, RA, Ross, JH, Rodriguez-Galindo, C & Frazier, AL 2018, 'Gonadal dysgenesis is associated with worse outcomes in patients with ovarian nondysgerminomatous tumors: A report of the Children's Oncology Group AGCT 0132 study', Pediatric Blood and Cancer. https://doi.org/10.1002/pbc.26913
Dicken, Bryan J. ; Billmire, Deborah F. ; Krailo, Mark ; Xia, Caihong ; Shaikh, Furqan ; Cullen, John W. ; Olson, Thomas A. ; Pashankar, Farzana ; Malogolowkin, Marcio H. ; Amatruda, James F. ; Rescorla, Frederick ; Egler, Rachel A. ; Ross, Jonathan H. ; Rodriguez-Galindo, Carlos ; Frazier, A. Lindsay. / Gonadal dysgenesis is associated with worse outcomes in patients with ovarian nondysgerminomatous tumors : A report of the Children's Oncology Group AGCT 0132 study. In: Pediatric Blood and Cancer. 2018.
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abstract = "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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T1 - Gonadal dysgenesis is associated with worse outcomes in patients with ovarian nondysgerminomatous tumors

T2 - A report of the Children's Oncology Group AGCT 0132 study

AU - Dicken, Bryan J.

AU - Billmire, Deborah F.

AU - Krailo, Mark

AU - Xia, Caihong

AU - Shaikh, Furqan

AU - Cullen, John W.

AU - Olson, Thomas A.

AU - Pashankar, Farzana

AU - Malogolowkin, Marcio H.

AU - Amatruda, James F.

AU - Rescorla, Frederick

AU - Egler, Rachel A.

AU - Ross, Jonathan H.

AU - Rodriguez-Galindo, Carlos

AU - Frazier, A. Lindsay

PY - 2018/1/1

Y1 - 2018/1/1

N2 - 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.

AB - 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.

KW - Gonadal dysgenesis

KW - Malignant ovarian germ cell tumor

KW - Pediatric outcome

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