The expression of
hormone receptors (HR) is considered a good prognostic marker in uterine
sarcoma. Hormonal
therapy is widely employed in the
therapy of HR positive breast and gynecologic
cancers, however, there is little information concerning hormonal
therapy in HR positive extrauterine
sarcoma. A 55-60 year age group female presented with an
estrogen receptor positive metastatic retroperitoneal
leiomyosarcoma (LMS). She was treated with four cycles of a combination of
Gemcitabine and
Paclitaxel.
Her disease remained stable for 29 months when
tamoxifen was initiated. The patient succumbed to an unrelated
malignancy after a total of 44 months of treatment. Despite emerging reports about the potential benefit of hormonal
therapy, selective
estrogen and
progesterone receptor modulators and
aromatase inhibitors, for uterine
sarcoma, there is a paucity of information regarding the application of these
therapies to
sarcomas arising at other sites. Our patient survived significantly longer than expected with metastatic retroperitoneal
sarcoma. In part this may be due to the survival benefit associated with HR positive
tumors, but it may also indicate a role for hormonal
therapy which has yet to be explored.