Abstract
The treatment of advanced uterine leiomyosarcomas (U-LMS) represents a considerable challenge. Radiological diagnosis prior to hysterectomy is difficult, with the diagnosis frequently made postoperatively. Whilst a total abdominal hysterectomy is the cornerstone of management of early disease, the role of routine adjuvant pelvic radiotherapy and adjuvant chemotherapy is less clear, since they may improve local tumor control in high risk patients but are not associated with an overall survival benefit. For recurrent or disseminated U-LMS, cytotoxic chemotherapy remains the mainstay of treatment. There have been few active chemotherapy drugs approved for advanced disease, although newer drugs such as trabectedin with its pleiotropic mechanism of actions represent an important addition to the standard front-line systemic therapy with doxorubicin and ifosfamide. In this review, we outline the therapeutic potential and in particular the emerging evidence-based strategy of therapy with trabectedin in patients with advanced U-LMS.
Highlights
Uterine leiomyosarcomas (U-LMS) are a group of rare and aggressive mesenchymal tumors, which comprise ∼1% of all uterine malignancies and a third of uterine sarcomas [1, 2]
While this study provides some evidence of efficacy, this data must be interpreted with caution since, in the absence of a no-treatment control group, the prolonged progression-free survival (PFS) cannot be attributed solely to the activity of the aromatase inhibitor treatment in this retrospective highly selected group of patients [76]
Standard treatment for early uterine leiomyosarcomas (U-LMS) is hysterectomy with bilateral salpingoophorectomy (BSO)
Summary
Uterine leiomyosarcomas (U-LMS) are a group of rare and aggressive mesenchymal tumors, which comprise ∼1% of all uterine malignancies and a third of uterine sarcomas [1, 2]. Several retrospective, nonrandomized studies had suggested an improved local control without demonstrating a significant survival benefit in patients with resected ULMS treated with adjuvant pelvic radiotherapy [20, 21]. This study was opened in 1988 and ran over a 13-year period to accrue a total of 224 patients with completely resected FIGO Stage I and II uterine sarcomas, including 103 patients with U-LMS. For those with U-LMS there was no benefit for radiotherapy for either diseasefree survival or OS. No benefit for adjuvant chemotherapy was found between groups neither for relapse-free survival nor for median OS and 5year OS rate (chemotherapy group: 66.5% versus observation control group: 67.8%)
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