RT Journal Article
SR Electronic
T1 69 Surgical and medical treatments for uterine PEComas
JF International Journal of Gynecologic Cancer
JO Int J Gynecol Cancer
FD BMJ Publishing Group Ltd
SP A151
OP A152
DO 10.1136/ijgc-2021-ESGO.250
VO 31
IS Suppl 3
A1 G Bogani
A1 A Gronchi
A1 A Ditto
A1 F Raspagliesi
YR 2021
UL http://ijgc.bmj.com/content/31/Suppl_3/A151.2.abstract
AB Introduction/Background*Perivascular epitheliod cell tumors (PEComas) are rare mesenchymal neoplasms. Uterine PEComa is extremely rare and only limited evidence is still available.Methodology This is a single-center retrospective study. Charts of consecutive patients who had treatment (from 01/01/2010 to 12/31/2020) for newly diagnosed uterine PEComas were retrieved. Five-year survival outcomes were assessed using Kaplan-Meier and Cox proportional hazard models.Result(s)*Data of 23 patients with newly diagnosed PEComas were analyzed. Mean (SD) patients‘ age was 52 (14) years. Twenty-two patients had a surgical cytoreductive attempt. In one case surgery was not performed due to the presence of an extra-abdominal spread. Overall, seven (30%) patients had disease outside the uterus at the time of surgery. Complete cytoreduction (no macroscopic residual tumor) was achieved in 19 patients. Complete cytoreduction was not completed in three patients who gross extrauterine disease and in the aforementioned patient who had not surgery. Eleven (48%) patients had adjuvant treatments, consisting in anthracycline-based chemotherapy (n=4),gemcitabine-based chemotherapy (n=2), mTOR inhibitors (n=4) and hormonal treatment (n=1). Median (range) follow-up as 23 (2, 99) months. Eleven (48%) recurrences occurred with a mean (SD) progression free-survival of 14 (11) months. After a median (range) follow-up of 23 (2, 99) months, nine (39%) patients died of disease. Residual tumor at surgery was the only factor correlating with the risk of developing recurrent disease (p=0.023) and worse overall survival (p=0.014). In our small series, stage of disease and adjuvant therapy administration had no impact on survival outcomes.Conclusion*Uterine PEComa represents a rare and aggressive entity. Molecular/genomic profiling of the disease is necessary to predict response to treatment. Further collaborative investigations are warranted to assess the role of various prognostic factors and evaluate the most effective surgical and medical treatment modalities.