Outcomes for 24 patients with metastatic MITF family translocation renal cell carcinoma treated with an immune checkpoint inhibitor (ICI)

Patient numberSexAge at diagnosis, yearsTranslocation typeTNM at diagnosisFISH analysisKarnofsky scoreHeng score (IDMC criteria)First treatment lineResponse to 1st linePFS (mo)Immunotherapy treatmentICI lineResponse to ICIDuration of response (mo)DeseasedSurvival (mo)
1F25TFE BT3N2M1available1002SunitinibPD2Ipilimumab4PR9.0no40
2F3TFE 3+TxN0M0available1001SunitinibPD1Ipilimumab5PD2.5yes23.5
3F13.7TFE 3+T2N + M+available603PazopanibPR4Ipilimumab3PD2,0yes20.7
4F10TFE 3+TxNxM0available< 801EverolimusPD4.4Atezolizumab2PD0.8no19
5F35.5TFE 3+T2bN1Mxavailable1004SunitinibPD4.4Nivolumab5PD1.0yes24.5
6M22TFE 3+pT1bNxM0available1001SunitinibPD1Nivolumab2PR8.3no17
7F26.7TFE 3+pT1bNxMxR1available1000SunitinibPD4Nivolumab3PD2.4no25
8M60.7TFE BpT3cN0M1R1available1001SunitinibPD3.1Nivolumab3PD2.5yes19.5
9F35TFE 3+pT3aN1Mxavailable1001SunitinibPD2.7Nivolumab2PD1.0yes9.6
10M41.9TFE 3+pT3bN1Mxno901PazopanibPD1.2Nivolumab2PD1.4yes8.5
11F35.1TFE 3+T3cN0M0available901High dose IL-2PD0.1MEDI4736 + Tremelimumab4PD2.4yes16.9
12F16.1TFE 3+T1aN1M1available702Sunitinib +nivolumabSD3.2Sunitinib +nivolumab1PR3.2yes7.6
13F79.5TFE 3+T3aN0M0available601TemsirolimusSD1.2Nivolumab5PD1.0yes17.3
14F54TFE 3+T2aN0M1available802PazopanibPR17.2Nivolumab2SD15.4no84.6
15F42.8TFE 3+T3N + M+available1001SunitinibSD22Nivolumab4PD9.0no59
16M32TFE BT3bN0M1available800SunitinibPD1Nivolumab2SD14no25
17F29.4TFE 3+T4N1M1available805PazopanibSD3Nivolumab2PD3no4
18F26.7TFE 3+NAno902SunitinibSD15pembro +41BB agonist3PR30yes60
19F49TFE 3+pT4pN1pM1noNA1SunitinibPD3Nivolumab2SD8.5yes19
20F43TFE 3+T3bN0M0available901SunitinibPD1.5Nivolumab3PD1yes8
21F63TFE 3+pT3aNxMxavailable901SunitinibPD6Nivolumab5PD2yes24
22F48.2TFE 3+T3NxM1available703SunitinibPD3Nivolumab2PD2no5
23F45TFE 3+pT3aNxM1R1available801SunitinibPD2Nivolumab2PD3yes9
24F24TFE 3+pT2bN1availableNA1SorafenibSD12Nivolumab3PD4no70

PR: partial response; SD: stable disease; PD: progressive disease