Document Detail

Aggressive Behavior and Poor Prognosis of Endometrial Stromal Sarcomas With YWHAE-FAM22 Rearrangement Indicate the Clinical Importance to Recognize This Subset.
MedLine Citation:
PMID:  25244606     Owner:  NLM     Status:  Publisher    
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.
Arnold-Jan Kruse; Sabrina Croce; Roy F P M Kruitwagen; Robert G Riedl; Brigitte F M Slangen; Toon Van Gorp; Koen K Van de Vijver
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-9-19
Journal Detail:
Title:  International journal of gynecological cancer : official journal of the International Gynecological Cancer Society     Volume:  -     ISSN:  1525-1438     ISO Abbreviation:  Int. J. Gynecol. Cancer     Publication Date:  2014 Sep 
Date Detail:
Created Date:  2014-9-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9111626     Medline TA:  Int J Gynecol Cancer     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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