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Treatment and recurrence patterns in endometrial stromal sarcomas and the relation to c-kit expression
Melissa A Geller1, Peter Argenta, William Bradley
1Department of Obstetrics and Gynecology and Women's Health, Division of Gynecologic Oncology, University of Minnesota, 420 Delaware Street SE, Minneapolis, MN 55455, USA.
Introduction:
Endometrial stromal sarcomas (ESS) are a rare gynecologic malignancy. The optimal management of this cancer remains unclear, although previous reports have failed to demonstrate a clear benefit to adjuvant chemotherapy or radiation. With the successful application of directed biological therapy in other sarcomas, a review of the behavior and biology of this disease is warranted.
Objectives:
To review outcomes and patterns of failure in patients with endometrial stromal sarcoma diagnosed over 31 years at our institution and the relationship to protooncogene c-kit expression.
Materials And Methods:
Hospital records and pathology were reviewed for 28 patients with endometrial stromal sarcomas [19 low-grade (LGESS) and 9 high-grade (HGESS)] treated between 1972 and 2003. Archival tissue samples from 16 patients were available and stained with CD 117 (c-kit) antibody (1:25 dilution). Staining intensity was graded 1+ to 3+ and distribution of the cellular staining as focal (10-30% of the cells), intermediate (30-60% of the cells), or diffuse (>60% of the cells). Positive tumors had more than 10% of cells comprising the neoplasm display immunoreactivity. RESULTS.: We found a significant difference in 5-year overall survival between LGESS and HGESS (P = 0.001). There was no significant difference in overall survival for patients with local versus advanced disease (P = 0.53) or in overall survival for those who underwent lymphadenectomy and those who did not (P = 0.92). 50% of patients received postoperative radiation with no difference in disease-free or overall survival (P = 0.68 and P = 0.53). Ten patients relapsed (36%, four HGESS and six LGESS). Seven of sixteen (43.8%) tumor samples expressed detectable c-kit. Five of seven (71%) were HGESS, and the other two (22%) were LGESS tumors. The median survival of patients with c-kit-positive versus c-kit-negative tumors was 12 and 47 months, respectively.
Conclusions:
This study confirms the superior overall prognosis of LGESS relative to HGESS, despite the similar rates of relapse. Although hard to assess, due to population heterogeneity and small numbers, adjuvant chemotherapy and radiation appear to be of limited benefit. Expression of c-kit was common, especially in high-grade lesions and may represent a potential therapeutic target.
Insights
Endometrial stromal sarcoma (ESS) prognosis is better for low-grade (LGESS) than high-grade (HGESS) types, with c-kit expression potentially targeting therapy. Adjuvant treatments showed limited benefit for these rare gynecologic cancers.
Area of Science:
- Gynecologic Oncology
- Surgical Pathology
- Molecular Diagnostics
Background:
- Endometrial stromal sarcoma (ESS) is a rare gynecologic malignancy with unclear optimal management.
- Previous studies suggest limited benefit from adjuvant chemotherapy or radiation.
- Biological therapies are successful in other sarcomas, warranting investigation into ESS behavior and biology.
Purpose of the Study:
- To review outcomes and patterns of failure in endometrial stromal sarcoma (ESS) patients over 31 years.
- To investigate the relationship between ESS outcomes and protooncogene c-kit expression.
Main Methods:
- Retrospective review of 28 ESS patients (19 LGESS, 9 HGESS) diagnosed between 1972 and 2003.
- Immunohistochemical staining for CD 117 (c-kit) on archival tissue samples from 16 patients.
- Analysis of survival data, relapse patterns, and correlation with c-kit expression and treatment modalities.
Main Results:
- Significant difference in 5-year overall survival between LGESS and HGESS (P=0.001).
- No significant difference in survival based on disease stage or lymphadenectomy.
- 43.8% of tumors expressed c-kit, predominantly in HGESS (71%); median survival was shorter for c-kit-positive vs. c-kit-negative tumors (12 vs. 47 months).
Conclusions:
- Low-grade ESS (LGESS) has a superior prognosis compared to high-grade ESS (HGESS), despite similar relapse rates.
- Adjuvant chemotherapy and radiation appear to offer limited benefit in ESS management.
- C-kit expression is common, particularly in high-grade lesions, suggesting it as a potential therapeutic target for endometrial stromal sarcoma.
