YWHAE-rearranged high-grade endometrial stromal sarcoma: Two-center case series and response to chemotherapy Journal Article
| Authors: | Hemming, M. L.; Wagner, A. J.; Nucci, M. R.; Chiang, S.; Wang, L.; Hensley, M. L.; George, S. |
| Article Title: | YWHAE-rearranged high-grade endometrial stromal sarcoma: Two-center case series and response to chemotherapy |
| Abstract: | Objectives YWHAE-rearranged high-grade endometrial stromal sarcoma (HG-ESS) is a rare, recently defined uterine sarcoma harboring t(10;17)(q22;p13) resulting in YWHAE-NUTM2A/B fusion. Chemotherapy sensitivity of metastatic YWHAE-rearranged HG-ESS is unknown. We reviewed the response to chemotherapy in women with YWHAE-rearranged HG-ESS to provide guidance for clinical management. Methods We retrospectively identified patients diagnosed with YWHAE-rearranged HG-ESS who received treatment for metastatic disease at our institutions. Cytogenetics or fluorescence in situ hybridization were performed in all cases to confirm rearrangement and, in conjunction with histopathology, a diagnosis of YWHAE-rearranged HG-ESS. Patient demographics, tumor histology, surgical procedures, radiation therapy, chemotherapy and treatment responses were collected. Results Seven patients were identified with YWHAE-rearranged HG-ESS and met criteria for inclusion in this study. The median age at diagnosis was 45 (range 42–47). All patients had undergone hysterectomy with bilateral salpingo-oophorectomy. FIGO stage at diagnosis was IVB in four patients and a single patient each at stage IIIB, II or I. Median follow-up for the cohort was 27 months (range 6–123). Six patients received anthracycline-based chemotherapy, with two of six achieving a complete radiologic response. One patient received gemcitabine and docetaxel, resulting in a partial response. For three patients who died from metastatic disease, survival from initial diagnosis was 33, 100 and 123 months. Conclusions For metastatic YWHAE-rearranged HG-ESS, prolonged disease control following diagnosis was seen, with notable responses to anthracycline-based therapy. This emphasizes the need for appropriate molecular testing of uterine mesenchymal malignancies and suggests that chemotherapy is an effective treatment option for metastatic YWHAE-rearranged HG-ESS. © 2017 Elsevier Inc. |
| Keywords: | sarcoma; anthracyclines; endometrial stromal sarcoma; uterine mesenchymal neoplasms |
| Journal Title: | Gynecologic Oncology |
| Volume: | 145 |
| Issue: | 3 |
| ISSN: | 0090-8258 |
| Publisher: | Elsevier Inc. |
| Date Published: | 2017-06-01 |
| Start Page: | 531 |
| End Page: | 535 |
| Language: | English |
| DOI: | 10.1016/j.ygyno.2017.03.021 |
| PROVIDER: | scopus |
| PMCID: | PMC5471810 |
| PUBMED: | 28390819 |
| DOI/URL: | |
| Notes: | Article -- Export Date: 3 July 2017 -- Source: Scopus |
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