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

Melissa K. McConechy, Anniina Färkkilä, Hugo M. Horlings, Aline Talhouk, Leila Unkila-Kallio, Hannah S. van Meurs, Winnie Yang, Nirit Rozenberg, Noora Andersson, Katharina Zaby, Saara Bryk, Ralf Bützow, Johannes B. G. Halfwerk, Marc J. van de Vijver, Marrije R. Buist, Gemma G. Kenter, Sara Y. Brucker, Bernhard Krämer, Annette Staebler, Maaike C. G. BleekerMarkku Heikinheimo, Stefan Kommoss, C. Blake Gilks, Mikko Anttonen, David G. Huntsman

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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
Original languageEnglish
Pages (from-to)djw134
JournalJournal of the National Cancer Institute
Issue number11
Early online date2016
Publication statusPublished - Nov 2016


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