A Retrospective Study From The Royal Marsden Hospital (Rmh) Of Patients With Malignant Perivascular Epithelioid Cell Tumors (Pecoma) Receiving Treatment With Sirolimus (Si) Or Temsirolimus (Tsi)

10038 Background: Perivascular epithelioid cell tumors (PEComas) are rare tumors driven by tuberous sclerosis complex gene mutations causing upregulation of mTOR. Two studies reporting a total of five patients (pts) have described the treatment of PEComa by mTOR inhibition. We report the outcome of 7 pts with PEComa treated with SI or TSI at RMH. Methods: A retrospective analysis was performed on all pts with PEComa referred to the Sarcoma unit at RMH between 2000 and 2011. Data collected included gender, performance status, site of primary tumour, tumour size, surgery, SI/TSI and SI/TSI dose, toxicity according to common toxicity criteria (CTC) version 2.0 and response by RECIST. Results: Five (71%) pts were female, median age was 46 years. Two (29%) had metastatic disease at baseline. Most common primary site was the gastrointestinal tract (29%). Six pts received SI, one TSI. Median treatment duration was 131 days (7-1135). TSI was given at 25 mg IV weekly. Median starting dose of SI was 3 mg daily (1-4...