A multi-institutional review of outcomes of endometrial stromal sarcoma☆
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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Featured poster presentation at the 36th Annual Meeting of the Society of Gynecologic Oncologists, Miami, Florida, March 2005.