Abstract 1546P
Background
Perivascular epitheliod cell tumors (PEComas) are rare mesenchymal neoplasms. Uterine PEComa is extremely rare and only limited evidence is still available.
Methods
This is a single-center retrospective study. Charts of consecutive patients who had treatment (between 01/01/2010 and 12/31/2020) for newly diagnosed uterine PEComas were retrieved. The Institutional review board of the Fondazione IRCCS Istituto Nazionale dei Tumori approved this study (68/2012). Five-year survival outcomes were assessed using Kaplan-Meier and Cox proportional hazard models.
Results
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 extra-uterine 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.
Conclusions
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.
Clinical trial identification
INT68/2012.
Editorial acknowledgement
Legal entity responsible for the study
Fondazione IRCCS Istituto Nazionale dei Tumori.
Funding
Has not received any funding.
Disclosure
All authors have declared no conflicts of interest.