Matthew L Hemming1, Andrew J Wagner1, Marisa R Nucci2, Sarah Chiang3, Lu Wang3, Martee L Hensley4, Suzanne George5. 1. Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA 02215, USA. 2. Division of Women's and Perinatal Pathology, Department of Pathology, Brigham and Women's Hospital, Boston, MA 02215, USA. 3. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA. 4. Gynecologic Medical Oncology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center, Weill Cornell Medical College, New York, NY 10065, USA. 5. Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA 02215, USA. Electronic address: suzanne_george@dfci.harvard.edu.
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 27months (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 123months. 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.
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 27months (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 123months. 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.
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