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.