Table 2.

Review of published extrarenal PEComas treated with mTOR inhibitors.

Case #	Tumor type/location	mTOR inhibitor	Dose	Duration	Response	Other drugs (combined with mTOR inhibitor)	TSC1/2 mutation/LOH	pS6 IHC	Reference
1	Retroperitoneal PEComa	sirolimus	8 mg daily	16 months	CR	-	TSC2	+	Wagner AJ et al. 201017
2	Uterine, metastatic PEComa	sirolimus	2–8 mg daily	6 months	SD, then progression	(added later) clarithromycin sorafenib	TSC1	+	Wagner AJ et al. 201017
3	Uterine, metastatic PEComa	temsirolimus	25 mg IV weekly	9+ months	CR	-	N/A	+	Italiano A et al. 201016
4	Uterine, metastatic PEComa	temsirolimus	25 mg IV weekly	22 weeks	PR, then progression	-	N/A	+	Italiano A et al. 201016
5	Retroperitoneal metastatic PEComa	temsirolimus	10mg IV weekly*	2 cycles (8 weeks)	progression	topotecan, bortezomib (NCT00770731)	N/A	N/A	Subbiah V et al. 201021
6	Retroperitoneal metastatic PEComa	everolimus	10 mg daily	2 cycles (8 weeks)	progression	figitumumab	N/A	N/A	Quek R et al. 201122
7	Retroperitoneal PEComa	sirolimus	4 mg daily	22+ months	CR	-	TSC2	+	case described in this report
8	Retroperitoneal PEComa	sirolimus	3 mg daily	16+ months	CR	-	TSC2	+	case described in this report
9	Small bowel PEComa / recurrent	sirolimus	4 mg daily	14+ months	PR	-	TSC2	-	case described in this report
10	Liver PEComa	everolimus	5 mg daily	2 months	CR (tumor resected after 2 months of treatment)	-	TSC2	+/−	case described in this report
11	Adrenal PEComa with metastases	sirolimus	1–4 mg daily	2 years	progression	-	not found	-	case described in this report

CR-complete response, PR-partial response, SD-stable disease; N/A not available.

Note: Renal PEComa from Wagner AJ et al. 2010 was not included.

^*V Subbiah, personal communication