Elsevier

Gynecologic Oncology

Volume 105, Issue 3, June 2007, Pages 630-634
Gynecologic Oncology

A multi-institutional review of outcomes of endometrial stromal sarcoma

https://doi.org/10.1016/j.ygyno.2007.01.031Get rights and content

Abstract

Objective.

To compare the clinical behavior and outcomes of low- and high-grade endometrial stromal sarcomas (LGESS and HGESS), respectively.

Methods.

Patients with endometrial stromal sarcoma from five different institutions were identified and reviewed for clinicopathologic variables, surgical management and outcomes. Statistical calculations including Chi-square, t-test and survival using the Kaplan–Meier method with the log rank test were performed.

Results.

One hundred and five patients were identified with 72 having LGESS, 31 with HGESS and 2 having unclassified tumors. The mean age was 50 years for patients with LGESS and 64 years for those with HGESS (p < 0.0001). In patients with LGESS, 68% (49 patients) had disease confined to the uterine corpus or cervix compared to 39% (12 patients) in HGESS (p = 0.002). The median overall survival was 53 months for HGESS and had not yet been reached in LGESS with 87.8% alive at 80 months (p < 0.0001). In HGESS patients with extrauterine disease, the presence of residual disease greater than 2 cm had a significant effect on median survival. Median survival was 52 months for those who underwent optimal cytoreduction versus 2 months for those with suboptimal residual disease (p = 0.007). The impact of cytoreduction was not seen in LGESS patients with extrauterine disease with 82.1% alive at 78 months.

Conclusions.

Low-grade and high-grade endometrial stromal sarcomas represent two distinct clinical entities and should be treated as such. Survival in patients with high-grade tumors appears to be related to amount of residual disease at the completion of initial surgery and would suggest the need for aggressive cytoreduction. The role of surgical staging and optimal adjuvant therapy remains unclear.

Introduction

Endometrial stromal sarcomas (ESS) are rare uterine malignancies, accounting for less than 1% of all uterine cancers and approximately 7–15% of all uterine sarcomas [1]. ESS is characterized by cells that resemble those of the endometrial stroma during the proliferative phase of the menstrual cycle and have traditionally been classified as either low-grade or high-grade. Tumors with a mitotic count of less than 10 mitoses/10 high power field are considered low-grade, while those with higher mitotic counts are considered high-grade [2]. Low-grade ESS (LGESS) are generally slow growing malignancies with an indolent clinical course, but with a tendency for late recurrences. High-grade ESS (HGESS) behave in a more aggressive manner with a poorer overall prognosis.

Standard management for both LGESS and HGESS consists of total hysterectomy and bilateral salpingo-oophorectomy; however, the importance of comprehensive surgical staging, including pelvic and para-aortic lymphadenectomy, remains debatable [3]. Investigators have reported a variable incidence of nodal metastases in patients with LGESS particularly in the setting of presumed early-stage disease [4], [5], [6], [7]. Furthermore, controversy also exists in terms of the role of surgical tumor cytoreduction in the setting of disseminated disease.

The optimal adjuvant therapy of ESS remains unclear. Echt and Piver suggested that adjuvant radiation therapy reduces the incidence of pelvic recurrence; however, overall survival does not appear to be improved [1], [8], [9], [10], [11]. The optimal chemotherapeutic regimen for the treatment of ESS also remains unclear, with administration generally reserved for advanced or recurrent high-grade ESS, as low-grade tumors do not appear to respond to chemotherapy [3], [9]. Finally, ESS tend to express progesterone receptors and appear to be responsive to hormonal manipulation with GNRH analogues, systemic progesterone therapy and aromatase inhibitors in the setting of adjuvant therapy, recurrent disease or as a palliative measure [12], [13].

The objective of our study was to compare clinical tumor behavior and patient outcomes based on the traditional designation of LGESS or HGESS, with particular attention to demographic variables, stage and impact of cytoreduction.

Section snippets

Materials and methods

Following IRB approval, hospital and clinical records of 105 patients were reviewed at five participating institutions to identify patients diagnosed with and treated for endometrial stromal sarcoma between 1972 and 2004. Contributing institutions included the University of Oklahoma, the University of Alabama at Birmingham, Washington University at St. Louis, the Ohio State University and the University of Minnesota. Patient records were reviewed for demographics, clinicopathologic variables,

Results

One-hundred and five patients with endometrial stromal sarcoma from five participating institutions were evaluated during the study period. Seventy-two patients had LGESS, 31 patients had HGESS and 2 patient's tumors were not classified as either. Early stage tumors, defined as stage I and II, were more common in patients with LGESS (68% versus 39%) with advanced stage disease more common in women with HGESS (61% versus 31%) (p = 0.002). A complete stage breakdown is present in Table 1. The mean

Discussion

Traditionally, ESS have been divided into LGESS and HGESS based on microscopic and pathologic findings which appear to accurately predict clinical behavior and outcomes [2]. Importantly, this division into LGESS and HGESS has been utilized clinically for many years. Recently, the pathologic designation between low-grade and high-grade has been challenged, as it is recognized that most tumors classified as high-grade do not actually warrant this designation, as they are formed primarily of

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