Uterine sarcoma: Clinico-pathological characteristics and outcome

Objectives:

To investigate the clinical and histopathological characteristics, with the prognostic factors, treatment outcome, pattern of relapse, and survival analysis of uterine sarcoma patient

Methods:

All patients with histologically proven uterine sarcoma were identified using the database at King Abdulaziz University Hospital, Jeddah, Saudi Arabia between January 2000 and December 2012.

Results:

A total of 36 patients with uterine sarcoma were reviewed. The median age of all patients was 57 years, and the mean age was 57.72±13.17 years. Carcinosarcoma was reported in 21 patients (58%), leiomyosarcoma in 7 (19%), undifferentiated endometrial sarcoma in 6 (17%), and rhabdomyosarcoma in 2 (6%). Approximately half of the patients were stages III and IV (28% and 25%), while 15 patients (41%) were stage I; only 2 patients (6%) were stage II. The surgical treatment was hysterectomy and bilateral salpingoophorectomy (H+BSO) plus staging in 18 patients (50%), while in 4 patients (19%), H+BSO plus debulking was performed. Adjuvant chemotherapy was given in 24 (69%) and adjuvant radiotherapy in 5 (14%) cases, At a median follow-up period of 13.5 months, 8 patients (22%) relapsed. The 2-year disease-free survival (DFS) rate was 22% and the 5-year was 14%. In the multivariate analysis, the advanced stages (p=0.015) and lymph vascular invasion (p=0.0001) were associated with poor DFS, while the use of chemotherapy significantly improved the DFS (p=0.027).

Conclusions:

The poor outcome of high-grade uterine sarcoma patients was identified, and only one third of patients (30%) survived for 2 years. This finding necessitates the need for more aggressive tools to fight this disease.Uterine sarcomas are rare tumors that account for 1-3% of all female genital tract malignancies and 3-8% of all uterine malignancies.1-3 This heterogeneous group of tumors originates from uterine mesodermal tissue. The major uterine sarcomas consist of leiomyosarcoma (LMS) and endometrial stromal tumors (EST), the latter of which are sub-classified into 3 categories by the World Health Organization (WHO): endometrial stromal nodules (ESNs), endometrial stromal sarcoma (ESS, historically referred to as low-grade sarcoma), and undifferentiated endometrial sarcoma (UES, historically called high-grade sarcoma). Carcinosarcoma, previously called malignant mixed mullerian tumor, is considered a deviant of carcinoma, and its behavior, and treatment resembles those of high-grade carcinoma. However, there are still some centers that treat this as sarcoma.4 Uterine sarcomas occur primarily in women who are 40-60 years old.5,6 A history of pelvic irradiation was also considered a risk factor in 5-10% of cases.7 Compared with the more common types of endometrial cancer, women with uterine sarcoma have a poor prognosis due to the aggressiveness of the disease.5-7 The most frequent prognostic factors include the stage, histological subtype, grade, lymph vascular invasion, and menopausal status.8-10 Standard treatment of early stage patients are hysterectomy and surgical staging, and approximately half of these patients develop recurrent disease.10 Post-operative radiotherapy reduces local recurrence and improves local disease but does not affect the overall survival.11,12 Adjuvant chemotherapy with a single agent, isofosamide or doxorubicin, has been used,11 and combination chemotherapy (which did not show any superiority) has non-proven value over a single agent.13 Due to its rarity, heterogeneity, and aggressiveness, there is no consensus regarding the optional therapeutic approaches with considerable variation in the type of surgery and choice of adjuvant treatment. The purpose of this study is to investigate the clinical and histopathological characteristics, with the prognostic factors, treatment outcome, and pattern of relapse and survival analysis of uterine sarcoma patients.