TY - JOUR T1 - Aggressive Behavior and Poor Prognosis of Endometrial Stromal Sarcomas With <em>YWHAE-FAM22</em> Rearrangement Indicate the Clinical Importance to Recognize This Subset JF - International Journal of Gynecologic Cancer JO - Int J Gynecol Cancer SP - 1616 LP - 1622 DO - 10.1097/IGC.0000000000000278 VL - 24 IS - 9 AU - Arnold-Jan Kruse AU - Sabrina Croce AU - Roy F.P.M. Kruitwagen AU - Robert G. Riedl AU - Brigitte F.M. Slangen AU - Toon Van Gorp AU - Koen K. Van de Vijver Y1 - 2014/11/01 UR - http://ijgc.bmj.com/content/24/9/1616.abstract N2 - Objectives Although the World Health Organization (WHO) in 2003 defined endometrial stromal sarcomas (ESSs) in general have a good prognosis, considerable differences in clinical behavior and prognosis may exist between different patients with ESS. The ESSs of the type associated with YWHAE-NUTM2 (previously named YWHAE-FAM22) fusion have a more aggressive clinical behavior and poorer prognosis than conventional ESS. Recently, the WHO 2014 classification recognizes this subset of ESS as a separate entity and classifies these as high-grade ESSs. Recognition of this subset has therefore an important clinical impact. We performed a review of the literature to delineate the clinicopathologic features of ESS patients with an YWHAE-NUTM2 rearrangement, with the goal to recognize this subset of ESS.Methods We report a case of a woman with WHO 2014–defined high-grade ESS. Furthermore, published English literature was reviewed for YWHAE-FAM22 ESS and uterus.Results Twenty patients were identified, with a median age of 50 (range, 28–67) years. There were no clinical features able to recognize YWHAE-NUTM2 ESS. However, they characteristically contain specific histopathological features. Furthermore, YWHAE-NUTM2 ESSs are strongly cyclin D1 positive in contrast to conventional low-grade ESSs.Conclusions YWHAE-NUTM2 ESSs represent a subset of ESSs with an aggressive clinical behavior and poor prognosis. Specific histopathological features may indicate the presence of YWHAE-NUTM2 rearrangement, which subsequently can be confirmed by cyclin D1 immunostaining. ER -