TY - JOUR
T1 - Molecularly Defined Adult Granulosa Cell Tumor of the Ovary: The Clinical Phenotype
T2 - The Clinical Phenotype
AU - McConechy, Melissa K.
AU - Färkkilä, Anniina
AU - Horlings, Hugo M.
AU - Talhouk, Aline
AU - Unkila-Kallio, Leila
AU - van Meurs, Hannah S.
AU - Yang, Winnie
AU - Rozenberg, Nirit
AU - Andersson, Noora
AU - Zaby, Katharina
AU - Bryk, Saara
AU - Bützow, Ralf
AU - Halfwerk, Johannes B. G.
AU - van de Vijver, Marc J.
AU - Buist, Marrije R.
AU - Kenter, Gemma G.
AU - Brucker, Sara Y.
AU - Krämer, Bernhard
AU - Staebler, Annette
AU - Bleeker, Maaike C. G.
AU - Heikinheimo, Markku
AU - Kommoss, Stefan
AU - Blake Gilks, C.
AU - Anttonen, Mikko
AU - Huntsman, David G.
PY - 2016/11
Y1 - 2016/11
N2 - 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
AB - 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
KW - Journal Article
U2 - https://doi.org/10.1093/jnci/djw134
DO - https://doi.org/10.1093/jnci/djw134
M3 - Article
C2 - 27297428
SN - 0027-8874
VL - 108
SP - djw134
JO - Journal of the National Cancer Institute
JF - Journal of the National Cancer Institute
IS - 11
ER -