RESUMO
Like other uterine sarcomas, low-grade endometrial stromal sarcomas (LG-ESS) are a very rare tumor entity. In the past, research studies therefore discussed the various different types of the disease in combination. In addition, the classification of endometrial stromal tumors presented difficulties for quite some time so that in earlier studies it was not always possible to precisely distinguish between LG-ESS, high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. For LG-ESS, surgery with hysterectomy and adnexectomy is the first-line treatment. The benefits of lymphadenectomy and tumor debulking are unclear. Endocrine therapy with gestagens and aromatase inhibitors is under discussion to provide adjuvant treatment for patients with advanced stages of the disease. As radiotherapy only provides locoregional control, and in view of the usually good prognosis of patients with LG-ESS, its benefits need to be weighed against its side effects. In the case of recurrence, repeat surgery is the first choice. Further research studies viewing LG-ESS as a distinct entity are needed in order to improve treatment options for patients with LG-ESS.
Assuntos
Neoplasias do Endométrio/terapia , Endométrio/patologia , Recidiva Local de Neoplasia/terapia , Sarcoma do Estroma Endometrial/terapia , Antineoplásicos Hormonais/uso terapêutico , Quimioterapia Adjuvante/métodos , Neoplasias do Endométrio/diagnóstico , Neoplasias do Endométrio/patologia , Endométrio/cirurgia , Feminino , Humanos , Histerectomia/métodos , Excisão de Linfonodo/métodos , Gradação de Tumores , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/patologia , Radioterapia Adjuvante/métodos , Ensaios Clínicos Controlados Aleatórios como Assunto , Reoperação/métodos , Salpingo-Ooforectomia/métodos , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma do Estroma Endometrial/patologiaRESUMO
PURPOSE: There is low evidence regarding the optimal treatment in patients with uterine sarcomas and malignant mixed Mullerian tumors (MMMTs). This study provides an overview of experience at our center with patients diagnosed with uterine sarcoma and MMMT, in relation to the clinical management and outcome. METHODS: The medical records for 143 patients with low-grade endometrial stromal sarcoma (ESS), leiomyosarcoma (LMS), and high-grade (undifferentiated) endometrial sarcoma (UES) and MMMT were reviewed. All available clinical and pathological data were collected and analyzed. Putative prognostic factors were entered into a multivariate analysis using a Cox proportional hazards ratio model, and survival data were calculated. RESULTS: The 5-year overall survival rates were significantly different between patients with ESS, LMS, and UES and MMMT (86 vs. 40 vs. 57 vs. 45 %; P < 0.001). The multivariate analysis showed that the patients' age, higher FIGO stage (III-IV), a history of smoking, prior pelvic radiation, diabetes, and residual tumor after surgery were associated with a poorer overall survival. Histological subtypes of LMS (HR 4.68; 95 % CI 1.35-16.17), UES (HR 1.21; 95 % CI 0.26-5.77) and MMMT (HR 1.63; 95 % CI 0.42-6.43) were also associated with a poorer overall survival than ESS (P = 0.008). Adjuvant therapies showed no associations with overall survival. CONCLUSIONS: Adjuvant therapy has so far not shown any overall survival benefit, and the focus is therefore on primary surgery. In future studies, the entities should be investigated separately in relation to prognostic factors and effective therapeutic management.
