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

The histopathologic features of Adult Granulosa Cell Tumors (AGCTs) are relatively non-specific, 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<0.001). 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, pre-molecular 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.

Article; Postprint

eng

Vancouver : University of British Columbia Library

10.14288/1.0314929

Medicine, Faculty of; Non UBC; Obstetrics and Gynaecology, Department of; Pathology and Laboratory Medicine, Department of

10.1093/jnci/djw134

Faculty; Postdoctoral; Graduate; Other

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