Outcomes after recurrence of low-grade endometrial stromal sarcoma (LGESS) (150)

Abstract

Objectives: To evaluate clinical characteristics of patients with recurrent low-grade endometrial sarcoma (LGESS) and identify prognostic factors associated with improved outcomes. Methods: All patients with a diagnosis of recurrent LGESS treated for their first recurrence at our institution between 1980 and 2019 were retrospectively identified. All recurrent uterine pathologies were reviewed, and pertinent clinical data were abstracted from the medical record. Survival outcomes were estimated using Kaplan-Meier method and differences in survival were analyzed using Logrank tests. Results: Among 28 patients with recurrent LGESS, the median time to first recurrence after the initial diagnosis was 49.5 months (range: 5.1-265.5). The median age at the time of recurrence was 54 years (range: 26-83). The sites of first recurrence were abdomen/pelvis (20, 71%), lung only (7, 25%), lung/spine (1, 4%). Eighteen patients (65%) had multi-site recurrent disease. The median follow-up time was 75.9 months (range: 8.3-232.7). Twenty-two patients (79%) experienced subsequent recurrence(s) with median progression-free survival (PFS) of 34.3 months (95% CI: 14.5-54.2). Median overall survival (OS) after the first recurrence was 136.2 months (95% CI: 87.4-185.0). At the time of the first recurrence, 21 patients (75%) underwent secondary surgical cytoreduction (SSR), and seven (25%) received non-surgical management alone. Reasons for non-surgical management included bony involvement (n=2), diffuse metastatic disease (n=2), short disease-free interval (DFI) (n=1), local extent of disease (n=1), and patient declining resection (n=1). Of those who underwent SSR, complete gross resection (CGR) was obtained in 15 (71%). CGR was not associated with PFS or OS, as most subsequent recurrences were salvaged. Seventeen of 21 (81%) SSR patients received postoperative therapy. ER+ and PR+ recurrent tumors and single-site recurrent disease were factors associated with a significant improvement in PFS (p=0.005 and p=0.03, respectively), but these did not impact OS (p=0.5 and p=0.2, respectively). An initial DFI of >4 years was associated with improved PFS (p=0.06) but no improvement in OS (p=0.1). Conclusions: Patients with a first recurrence of LGESS are likely to recur again. Longer DFI, ER+/PR+ tumors and single-site disease are associated with greater time to subsequent recurrence. Outcomes are generally favorable with this rare tumor, and all multi-modality therapy options should be considered for salvage, including surgical resection. Objectives: To evaluate clinical characteristics of patients with recurrent low-grade endometrial sarcoma (LGESS) and identify prognostic factors associated with improved outcomes. Methods: All patients with a diagnosis of recurrent LGESS treated for their first recurrence at our institution between 1980 and 2019 were retrospectively identified. All recurrent uterine pathologies were reviewed, and pertinent clinical data were abstracted from the medical record. Survival outcomes were estimated using Kaplan-Meier method and differences in survival were analyzed using Logrank tests. Results: Among 28 patients with recurrent LGESS, the median time to first recurrence after the initial diagnosis was 49.5 months (range: 5.1-265.5). The median age at the time of recurrence was 54 years (range: 26-83). The sites of first recurrence were abdomen/pelvis (20, 71%), lung only (7, 25%), lung/spine (1, 4%). Eighteen patients (65%) had multi-site recurrent disease. The median follow-up time was 75.9 months (range: 8.3-232.7). Twenty-two patients (79%) experienced subsequent recurrence(s) with median progression-free survival (PFS) of 34.3 months (95% CI: 14.5-54.2). Median overall survival (OS) after the first recurrence was 136.2 months (95% CI: 87.4-185.0). At the time of the first recurrence, 21 patients (75%) underwent secondary surgical cytoreduction (SSR), and seven (25%) received non-surgical management alone. Reasons for non-surgical management included bony involvement (n=2), diffuse metastatic disease (n=2), short disease-free interval (DFI) (n=1), local extent of disease (n=1), and patient declining resection (n=1). Of those who underwent SSR, complete gross resection (CGR) was obtained in 15 (71%). CGR was not associated with PFS or OS, as most subsequent recurrences were salvaged. Seventeen of 21 (81%) SSR patients received postoperative therapy. ER+ and PR+ recurrent tumors and single-site recurrent disease were factors associated with a significant improvement in PFS (p=0.005 and p=0.03, respectively), but these did not impact OS (p=0.5 and p=0.2, respectively). An initial DFI of >4 years was associated with improved PFS (p=0.06) but no improvement in OS (p=0.1). Conclusions: Patients with a first recurrence of LGESS are likely to recur again. Longer DFI, ER+/PR+ tumors and single-site disease are associated with greater time to subsequent recurrence. Outcomes are generally favorable with this rare tumor, and all multi-modality therapy options should be considered for salvage, including surgical resection.