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Endometrial stromal sarcoma – the new genetic paradigm
Author(s) -
Lee ChengHan,
Nucci Marisa R
Publication year - 2015
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.12594
Subject(s) - endometrial stromal sarcoma , sarcoma , stroma , stromal cell , biology , fusion gene , cancer research , pathology , medicine , gene , immunohistochemistry , genetics
Endometrial stromal sarcoma ( ESS ) is a gynaecological sarcoma that is composed of cells that resemble those of proliferative‐phase endometrial stroma. The 2014 World Health Organization tumour classification system separates ESS into low‐grade and high‐grade types, which are histologically, genetically and clinically distinct from undifferentiated uterine sarcoma ( UUS ). Low‐grade ESS s frequently contain chromosomal rearrangements that result in JAZF 1– SUZ 12 fusion or equivalent genetic fusions. Although most low‐grade ESS s show classic histological features that closely resemble those of proliferative‐phase endometrial stroma, there are several histological variants that are associated with the same genetic fusions as seen in the classic type. High‐grade ESS is defined by the presence of YWHAE – NUTM 2A/B ( YWHAE – FAM 22A/B ) fusions. High‐grade ESS s are clinically more aggressive than low‐grade ESS s, but are associated with a lower mortality rate than UUS s. The histological and immunophenotypic features of these different types of ESS , and their diagnostic considerations, are the subjects of this review.