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J Natl Cancer Inst. 2016 Jun 13;108(11). pii: djw134. doi: 10.1093/jnci/djw134. Print 2016 Nov.

Molecularly Defined Adult Granulosa Cell Tumor of the Ovary: The Clinical Phenotype.

Author information

1
Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver General Hospital and BC Cancer Agency, Vancouver, Canada (MKM, HMH, AT, WY, NR, CBG, DGH); Biomedicum Helsinki, University of Helsinki, Helsinki, Finland (AF, LUK, SB, MA); Department of Obstetrics and Gynecology, Helsinki University Hospital, Helsinki, Finland (AF, LUK, SB, MA); Centre of Gynaecologic Oncology Amsterdam (HSvM, MRB, GGK) and Department of Pathology (JBGH, GKJH, MJvdV, MCGB), Academic Medical Center, Amsterdam, the Netherlands; Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland (NA, MH); Department of Obstetrics and Gynaecology, Tübingen University Hospital, Department of Women's Health, Tübingen, Germany (KZ, SYB, BK, SK); Department of Pathology (RB) and Department of Clinical Chemistry and Hematology (MA), Helsinki University Hospital, University of Helsinki and HUSLAB, Helsinki, Finland; Institute of Pathology, Tübingen University Hospital, Tübingen, Germany (AS); Department of Pediatrics, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, MO (MH); Department of Obstetrics and Gynaecology, University of British Columbia, Vancouver, BC, Canada (DGH); Department of Human Genetics, Research Institute of the McGill University Health Centre, McGill University, Montreal, QC, Canada (MKM).
2
Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver General Hospital and BC Cancer Agency, Vancouver, Canada (MKM, HMH, AT, WY, NR, CBG, DGH); Biomedicum Helsinki, University of Helsinki, Helsinki, Finland (AF, LUK, SB, MA); Department of Obstetrics and Gynecology, Helsinki University Hospital, Helsinki, Finland (AF, LUK, SB, MA); Centre of Gynaecologic Oncology Amsterdam (HSvM, MRB, GGK) and Department of Pathology (JBGH, GKJH, MJvdV, MCGB), Academic Medical Center, Amsterdam, the Netherlands; Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland (NA, MH); Department of Obstetrics and Gynaecology, Tübingen University Hospital, Department of Women's Health, Tübingen, Germany (KZ, SYB, BK, SK); Department of Pathology (RB) and Department of Clinical Chemistry and Hematology (MA), Helsinki University Hospital, University of Helsinki and HUSLAB, Helsinki, Finland; Institute of Pathology, Tübingen University Hospital, Tübingen, Germany (AS); Department of Pediatrics, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, MO (MH); Department of Obstetrics and Gynaecology, University of British Columbia, Vancouver, BC, Canada (DGH); Department of Human Genetics, Research Institute of the McGill University Health Centre, McGill University, Montreal, QC, Canada (MKM) dhuntsma@bccancer.bc.ca anniina.farkkila@helsinki.fi.

Abstract

The histopathologic features of adult granulosa cell tumors (AGCTs) are relatively nonspecific, resulting in misdiagnosis of other cancers as AGCT, a problem that has not been well characterized. FOXL2 mutation testing was used to stratify 336 AGCTs from three European centers into three categories: 1) FOXL2 mutant molecularly defined AGCT (MD-AGCT) (n = 256 of 336), 2) FOXL2 wild-type AGCT (n = 17 of 336), 3) misdiagnosed other tumor types (n = 63 of 336). All statistical tests were two-sided. The overall and disease-specific survival of the misdiagnosed cases was lower than in the MD-AGCTs (P < .001). The misdiagnosed cases accounted for 71.9% of disease-specific deaths within five years. In the population-based cohort, overall survival of MD-AGCT patients was not different from age-matched, population-based controls. Even though 35.2% of all the MD-AGCT patients in our study experienced a relapse, AGCT is usually an indolent disease. The historical, premolecular data underpinning our clinical understanding of AGCT was likely skewed by inclusion of misdiagnosed cases, and future management strategies should reflect the potential for surgical cure and long survival even after relapse.

PMID:
27297428
PMCID:
PMC5241905
DOI:
10.1093/jnci/djw134
[Indexed for MEDLINE]
Free PMC Article

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