Abstract
Context: Endometrial stromal sarcomas (ESSs) are rare mesenchymal tumors of uterus which accounts for less
than 10% of all uterine sarcomas. Gene rearrangement studies unveil their genetic heterogeneity with the
discovery of specific YWHAE-FAM22 gene fusion. According to recent WHO classification of endometrial stromal
tumors all Endometrial stromal sarcomas with this fusion are labeled as high grade-Endometrial Stromal
Sarcomas (HG-ESS). Immunohistochemically HG-ESSs with YWHAE- FAM22 translocation express nuclear
Cyclin D1 positivity which can be used as a surrogate immunohistochemical marker for this translocation.
Objective: Determine diagnostic utility of Cyclin D1 as a marker to segregate high grade- Endometrial Stromal
Sarcomas (HG-ESS) from low grade-Endometrial Stromal Sarcomas (LG-ESS) and undifferentiated uterine
sarcoma (UUS) and from other uterine sarcomas including leiomyosarcomas and carcinosrcomas.
Materials and Methods: Cases of Endometrial stromal sarcomas, leiomyosarcomas and carcinosarcomas
received from Jan 2010- Jan 2015 were reviewed and immunostained with Cyclin D1, CD-10, ER, PR and Desmin.
Nuclear positive or negative staining for Cyclin D1 was noted.
Results: Total 36 cases of uterine sarcomas including 10 cases of ESS, 20 cases of leiomyosrcoma and 6 cases of
carcinosarcoma were retrieved from surgical database. Out of ten cases of endometrial stromal sarcomas, five
were low grade, two were high grade and three were undifferentiated sarcomas. Cyclin D1 was expressed in
three cases previously diagnosed as low grade, high grade and undifferentiated sarcomas respectively. Cyclin D1
positive undifferentiated uterine sarcoma (UUS) also revealed diffuse CD-10 positivity. Other two cases with a
positive Cyclin D1 expression revealed a patchy CD-10 positivity. Negative expression of Cyclin D1 was noted in
all cases of leiomyosrcoma and carcinosarcoma.
Conclusions: Endometrial stromal sarcomas are relatively uncommon tumors and can pose diagnostic problems
for pathologists. Smooth muscle tumors (leiomysrcomas) and carcinosarcomas are among the top differentials.
Final diagnosis usually requires immunohistochemistochemical analysis with a panel of immunomarkers. Cyclin
D1 expression along with patchy CD-10 positivity favors HG-ESS over LG-ESS and UUS. Cyclin D1 positivity
along with diffuse CD-10 expression favors Undifferentiated Uterine Sarcoma. Negative expression in other
uterine sarcomas makes it a specific marker for ESSs. It is suggested that Cyclin D1 along with CD-10, ER, PR and
Desmin should be added in immunohistochemical panel of high grade uterine sarcomas especially if patchy
expression of CD-10 is noted. It is to make a provisional diagnosis of YWHAE-FAM22 associated HG-ESS when
fluorescence in situ hybridization (FISH) and reverse transcriptase polymerase chain reaction (RT-PCR) are not
available to make a final diagnosis.
Dr. Kanwal Zahra, Ahmareen Khalid, Shaista Khurshid, Ghazal Zafar, Ashok Kumar Tanwani. (2017) Cyclin-D1 Utility in Classification of Endometrial Stromal Sarcomas and Segregation from other Uterine Sarcomas, International Journal of Pathology, Vol-15, Issue 2.
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