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Erschienen in:
20.11.2017 | Review
Is fertility-preservation safe for adult non-metastatic gynecologic rhabdomyosarcoma patients? Systematic review and pooled survival analysis of 137 patients
Erschienen in: Archives of Gynecology and Obstetrics | Ausgabe 3/2018
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Purpose
Recently, conservative approaches such as wide local excisions and neoadjuvant chemotherapy are being considered to select young adult females with gynecologic RMS who have strong desire to preserve fertility. This analysis aims to identify prognosticators affecting survival outcomes and defining potential candidacy for fertility-preservation. Another focus is to explore the role of chemotherapy in reducing the need for aggressive surgery and the role of radiotherapy in decreasing rates of local failure.
Methods
A comprehensive database search identified 137 females > 16 years old with primary non-metastatic gynecologic RMS, who were included in a multivariate survival analysis.
Results
5-year overall survival rate was 65%. Patients < 50 years old, with cervix uteri primaries, well-defined/polypoid presentations, embryonal histology and superficial tumors were more likely to survive. Deeply invasive disease and alveolar/pleomorphic histology significantly increased risks of treatment failure and tumor recurrence. Chemotherapy use was a significant multivariate predictor of better overall and metastasis-free survival. Radical surgery did not add local control or overall survival benefit for patients with superficial lesions (minimal/no cervical stromal invasion and no myometrial invasion).
Conclusions
While high-quality clinical trial evidence is missing, existing evidence seems to support holding back on radical surgery for selected candidates with well-defined, polypoid, superficial, embryonal cervical/endometrial RMS lesions that could be completely excised with conservative surgery; further local resections with/without radiotherapy are then warranted based on margin status. Experience on the use of neoadjuvant chemotherapy in the conservative management of uterine RMS in adults is very limited, though this approach is golden-standard in pediatrics. A suggested scheme is introduced for the management of uterine RMS.