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- Title
CAP-ACP Junior Scientist Award Lecture 2013: Molecular Classification of Endometrial Sarcoma.
- Authors
Cheng-Han Lee
- Abstract
Endometrial stromal sarcomas (ESSs) are traditionally classified into low-grade and high-grade groups. The diagnostic category of high-grade ESS was removed from the 2003 World Health Organization tumour classification system and replaced by undifferentiated endometrial sarcoma (UES) in recognition of the fact that many tumours defined as high-grade ESS were pleomorphic sarcomas that bear no resemblance to endometrial stromal tissue. Consequently, tumours that were previously considered as high-grade ESS have to be reclassified as low-grade ESS or UES. Recent advances in our understanding of the genetics of ESS, however, provide strong support for the existence of a group of histologically high-grade ESS, which is characterized by YWHAE-NUTM2 genetic fusion resulting from the translocation t(10;17)(q22;pl3). High-grade ESS with YWHAE-NUTM2 fusion shows more frequent recurrence and is associated with decreased survival compared to low-grade ESS (characterized most commonly by JAZF1-SUZ12 fusion). It does, however, show significantly better diseasespecific survival compared to UES. The histological, immunophenotypical, and genetic features of low-grade ESS, high-grade ESS, and UES, with emphasis on the diagnostic distinction between these clinically different tumour types, are reviewed.
- Subjects
DIAGNOSIS of endometrial cancer; TUMOR diagnosis; POLYMORPHISM (Crystallography); HISTOPATHOLOGY; IMMUNOPHENOTYPING; HEALTH outcome assessment; WORLD Health Organization
- Publication
Canadian Journal of Pathology, 2014, Vol 6, Issue 2, p36
- ISSN
1918-915X
- Publication type
Article