Discussion
Considering that the definitive treatment of metastatic euLMS, as a curative method, is still unknown and the rarity of these cases, there is urgent to provide such case reports 5. The Surgical resection of primary tumors that performed in more than 80% of all metastatic euLMS doesn’t seem to prolong their survival2. To reach the acceptable survival time, surgical resection of primary tumors and metastasectomy should be considered, based on the decision of multidisciplinary team during and after surgery, our case underwent surgical resection of metastatic tumors and adjuvant gemcitabine plus docetaxel chemotherapy started as soon as possible regarding the extent of the invasion.
Prognostic factors in metastatic extrauterine LMS (euLMS) are not completely explained, and it is not fully known that the choice of systemic therapy agent influence response rate 2,9. A large-scale retrospective study evaluated the association of clinicopathologic factors, first-line systemic therapy agent, and objective response with overall survival since metastasis in patients with advanced euLMS, they reported that younger age, female sex, smaller primary tumor size (<10 cm), and less than two sites of metastasis at the onset of advanced disease were independent predictors associated with better outcome 5.
Various chemotherapy regimens used in metastatic euLMS, include: doxorubicin, doxorubicin plus ifosfamide, gemcitabine plus docetaxel, pazopanib, eribulin and trabectedin. in addition, the number of chemotherapy cycle in these patients was in the range of 1 to 5 10. We used docetaxel plus gemcitabin chemotherapy regimen and continued for 4 cycles.
The most common sites of uLMS metastasis include, lung (74%), peritoneum (41%), Bones (33%) and liver (27%), however pancreas and gut (less than 1%) are uncommon metastatic sites11,12,13. In the present case, omentum, small intestine, recto-sigmoid colon, vermiform appendix and pancreas were under invasion. Based on a cohort study, patients with objective response to the first systemic treatment had better overall survival than cases with stable or primary progressive disease, therefore it is important to choose the best therapies which may be useful for patients with advanced euLMS5. A particular regimen cannot be found equally effective in all patients, the metastatic euLMS patients needs a multidisciplinary approach to select best therapies that may be beneficial for them.
more extensive investigations are needed to understand genetic and epigenetic factors influencing response rate of euLMS to chemotherapy regimen 14,15. The heterogeneity in molecular and immunological properties of euLMS also impact on clinical outcomes, there is a need to personalize treatment strategies to help agent sensitivity and longevity of patients with advanced disease5,16,17.