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Pediatr Blood Cancer. 2018 Apr;65(4). doi: 10.1002/pbc.26913. Epub 2017 Dec 29.

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

Author information

1
Stollery Children's Hospital, University of Alberta Hospital, Edmonton, Alberta, Canada.
2
Riley Hospital for Children, Indianapolis, Indiana.
3
Keck School of Medicine, University of Southern California, Los Angeles, California.
4
The Hospital for Sick Children, Toronto, Ontario, Canada.
5
Rocky Mountain Hospital for Children-Presbyterian St Luke's Medical Center, Denver, Colorado.
6
Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia.
7
Yale University, New Haven, Connecticut.
8
University of California Davis, Davis, California.
9
University of Texas Southwestern Medical Center, Dallas, Texas.
10
Rainbow Babies and Children's Hospital, Cleveland, Ohio.
11
St. Jude Children's Research Hospital, Memphis, Tennessee.
12
Dana-Farber Cancer Institute and Boston Children's Hospital, Boston, Massachusetts.

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.

KEYWORDS:

gonadal dysgenesis; malignant ovarian germ cell tumor; pediatric outcome

PMID:
29286555
PMCID:
PMC6219870
DOI:
10.1002/pbc.26913
[Indexed for MEDLINE]
Free PMC Article

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