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ZC3H7B-BCOR high-grade endometrial stromal sarcoma may present as myoma nascens with cytoplasmic signet ring cell change
O. Ondič, B. Bednářová, N. Ptáková, V. Hájková, P. Šteiner, H. Šidlová, J. Presl, J. Bouda, R. Alaghehbandan, M. Michal,
Language English Country Germany
Document type Case Reports, Journal Article
NLK
ProQuest Central
from 2003-01-01 to 1 year ago
Medline Complete (EBSCOhost)
from 2011-01-01 to 1 year ago
Nursing & Allied Health Database (ProQuest)
from 2003-01-01 to 1 year ago
Health & Medicine (ProQuest)
from 2003-01-01 to 1 year ago
- MeSH
- Sarcoma, Endometrial Stromal genetics pathology MeSH
- Carcinoma, Signet Ring Cell diagnosis pathology MeSH
- Middle Aged MeSH
- Humans MeSH
- Biomarkers, Tumor genetics MeSH
- Uterine Neoplasms pathology MeSH
- Endometrial Neoplasms genetics pathology MeSH
- RNA-Binding Proteins metabolism MeSH
- Proto-Oncogene Proteins genetics metabolism MeSH
- Repressor Proteins metabolism MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
We report on 51-year-old woman who presented with brown discharge and postcoital bleeding due to myoma nascens-like polypoid mass distending cervical canal. Histologically, the tumor consisted of high-grade spindle cell component with up to 15 mitotic figures per 10 HPF and also low-grade leiomyoma-like areas with focal myxoid change and so far undescribed cytoplasmic signet ring cell change. Immunohistochemically Desmin, actin, and h-caldesmon were negative. Conversely, BCOR positive expression was coupled with Cyclin D1 positivity and was antibody clone dependent. The molecular NGS and FISH study identified reciprocal fusion gene ZC3H7B-BCOR. In conclusion, these findings further support the idea of routine reflex molecular testing of uterine mesenchymal tumors with unusual clinical presentation or in case malignancy is suspected. Lastly, we suggest ZC3H7B-BCOR rearranged high-grade endometrial stromal sarcoma might be considered as a tumor suitable for BCL6-targeted treatment.
Bioptická laboratoř s r o Pilsen Czech Republic
Department of Pathology Slovak Medical University in Bratislava Bratislava Slovak Republic
Department of Pathology University of British Columbia Royal Columbian Hospital Vancouver BC Canada
References provided by Crossref.org
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- $a We report on 51-year-old woman who presented with brown discharge and postcoital bleeding due to myoma nascens-like polypoid mass distending cervical canal. Histologically, the tumor consisted of high-grade spindle cell component with up to 15 mitotic figures per 10 HPF and also low-grade leiomyoma-like areas with focal myxoid change and so far undescribed cytoplasmic signet ring cell change. Immunohistochemically Desmin, actin, and h-caldesmon were negative. Conversely, BCOR positive expression was coupled with Cyclin D1 positivity and was antibody clone dependent. The molecular NGS and FISH study identified reciprocal fusion gene ZC3H7B-BCOR. In conclusion, these findings further support the idea of routine reflex molecular testing of uterine mesenchymal tumors with unusual clinical presentation or in case malignancy is suspected. Lastly, we suggest ZC3H7B-BCOR rearranged high-grade endometrial stromal sarcoma might be considered as a tumor suitable for BCL6-targeted treatment.
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