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

• Type of Material: Article
• Authors: Bryan J. Dicken, University of Alberta Hospital; Deborah F. Billmire, Riley Hospital for Children; Mark Krailo, University of Southern California; Caihong Xia, University of Southern California; Furqan Shaikh, Hospital for Sick Children; John W. Cullen, Rocky Mountain Hospital for Children; Thomas Olson, Emory University; Farzana Pashankar, Yale University; Marcio H. Malogolowkin, University of California Davis; James F. Amatruda, University of Texas Southwestern Medical Center of Dallas; Frederick J. Rescorla, Riley Hospital for Children; Rachel A. Egler, Rainbow Babies and Children's Hospital; Jonathan H. Ross, Rainbow Babies and Children's Hospital; Carlos Rodriguez-Galindo, St Jude Childrens Research Hospital; A. Lindsay Frazier, Dana Farber Cancer Institute
• Language: English
• Date: 2018-04-01
• Publisher: Wiley: 12 months
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2017 Wiley Periodicals, Inc.
• Final Published Version (URL): http://dx.doi.org/10.1002/pbc.26913
• Title of Journal or Parent Work: Pediatric Blood and Cancer
• ISSN: 1545-5009
• Volume: 65
• Issue: 4
• Start Page: e26913
• End Page: e26913
• Keywords: GONADOBLASTOMA; MALIGNANCY; Oncology; Y-CHROMOSOME; GERM-CELL TUMORS; malignant ovarian germ cell tumor; DISORDERS; RISK; Life Sciences & Biomedicine; Hematology; SARCOMATOUS COMPONENTS; SEX DEVELOPMENT; PROPHYLACTIC GONADECTOMY; Pediatrics; pediatric outcome; gonadal dysgenesis; Science & Technology; PHENOTYPIC FEMALE-PATIENTS
• Research Categories: Health Sciences, Oncology

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.