Feng Ling1, Sibei Ruan1, Huiling Chen2, Xiaoming Xiong1, Dongmei Zhao3, Johannes Haybaeck4,5, Cuiwei Zhang6. 1. Department of Pathology, The Affiliated Hospital of Southwest Medical University, Luzhou, P.R. China. 2. School of clinical medicine, Southwest Medical University, Luzhou, P.R. China. 3. Department of Radiology, The Affiliated Hospital of Southwest Medical University, Luzhou, P.R. China. 4. Institute of Pathology, Neuropathology and Molecular Pathology, Medical University of Innsbruck, Innsbruck, Austria; johannes.haybaeck@i-med.ac.at zcw.37@163.com. 5. Diagnostic and Research Center for Molecular Biomedicine, Institute of Pathology, Medical University of Graz, Graz, Austria. 6. Department of Pathology, The Affiliated Hospital of Southwest Medical University, Luzhou, P.R. China; johannes.haybaeck@i-med.ac.at zcw.37@163.com.
Abstract
BACKGROUND: The coexistence of a uterine leiomyosarcoma and a high-grade endometrial stromal sarcoma (HGESS) is extremely rare, especially when one of the components causes metastasis. CASE REPORT: A 46-year-old female with aggravated abdominal pain for more than 4 months was diagnosed with uterine malignant mesenchymal tumor composed of predominantly a leiomyosarcoma (99%) and a minor component of HGESS with BCL6 corepressor (BCOR) gene alterations (1%), with ovarian and pelvic metastases. RESULTS: The volume of HGESS with BCOR gene alterations accounted for less than 1% of the tumor mass but caused ovarian and pelvic metastases. CONCLUSION: HGESS with BCOR gene alterations is extremely aggressive. We suggest that when both components of HGESS with BCOR gene alterations and uterine leiomyosarcoma are present in one patient, the HGESS with BCOR gene alterations needs to be highlighted in the pathological report, even if it accounts for less than 1% of the tumor volume.
BACKGROUND: The coexistence of a uterine leiomyosarcoma and a high-grade endometrial stromal sarcoma (HGESS) is extremely rare, especially when one of the components causes metastasis. CASE REPORT: A 46-year-old female with aggravated abdominal pain for more than 4 months was diagnosed with uterine malignant mesenchymal tumor composed of predominantly a leiomyosarcoma (99%) and a minor component of HGESS with BCL6 corepressor (BCOR) gene alterations (1%), with ovarian and pelvic metastases. RESULTS: The volume of HGESS with BCOR gene alterations accounted for less than 1% of the tumor mass but caused ovarian and pelvic metastases. CONCLUSION: HGESS with BCOR gene alterations is extremely aggressive. We suggest that when both components of HGESS with BCOR gene alterations and uterine leiomyosarcoma are present in one patient, the HGESS with BCOR gene alterations needs to be highlighted in the pathological report, even if it accounts for less than 1% of the tumor volume.
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