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Aggressive Behavior and Poor Prognosis of Endometrial Stromal Sarcomas With YWHAE-FAM22 Rearrangement Indicate the Clinical Importance to Recognize This Subset

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Abstract
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 anYWHAE-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.

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Chicago
Kruse, Arnold-Jan, Sabrina Croce, Roy F. P. M. Kruitwagen, Robert G. Riedl, Brigitte F. M. Slangen, Toon Van Gorp, and Koen Van de Vijver. 2014. “Aggressive Behavior and Poor Prognosis of Endometrial Stromal Sarcomas With YWHAE-FAM22 Rearrangement Indicate the Clinical Importance to Recognize This Subset.” International Journal of Gynecological Cancer 24 (9): 1616–1622.
APA
Kruse, A.-J., Croce, S., Kruitwagen, R. F. P. M., Riedl, R. G., Slangen, B. F. M., Van Gorp, T., & Van de Vijver, K. (2014). Aggressive Behavior and Poor Prognosis of Endometrial Stromal Sarcomas With YWHAE-FAM22 Rearrangement Indicate the Clinical Importance to Recognize This Subset. INTERNATIONAL JOURNAL OF GYNECOLOGICAL CANCER, 24(9), 1616–1622.
Vancouver
1.
Kruse A-J, Croce S, Kruitwagen RFPM, Riedl RG, Slangen BFM, Van Gorp T, et al. Aggressive Behavior and Poor Prognosis of Endometrial Stromal Sarcomas With YWHAE-FAM22 Rearrangement Indicate the Clinical Importance to Recognize This Subset. INTERNATIONAL JOURNAL OF GYNECOLOGICAL CANCER. 2014;24(9):1616–22.
MLA
Kruse, Arnold-Jan, Sabrina Croce, Roy F. P. M. Kruitwagen, et al. “Aggressive Behavior and Poor Prognosis of Endometrial Stromal Sarcomas With YWHAE-FAM22 Rearrangement Indicate the Clinical Importance to Recognize This Subset.” INTERNATIONAL JOURNAL OF GYNECOLOGICAL CANCER 24.9 (2014): 1616–1622. Print.
@article{8587767,
  abstract     = {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 anYWHAE-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.},
  author       = {Kruse, Arnold-Jan and Croce, Sabrina and Kruitwagen, Roy F. P. M. and Riedl, Robert G. and Slangen, Brigitte F. M. and Van Gorp, Toon and Van de Vijver, Koen},
  issn         = {1048-891X},
  journal      = {INTERNATIONAL JOURNAL OF GYNECOLOGICAL CANCER},
  number       = {9},
  pages        = {1616--1622},
  title        = {Aggressive Behavior and Poor Prognosis of Endometrial Stromal Sarcomas With YWHAE-FAM22 Rearrangement Indicate the Clinical Importance to Recognize This Subset},
  url          = {http://dx.doi.org/10.1097/IGC.0000000000000278},
  volume       = {24},
  year         = {2014},
}

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