| Author | Therapy | Patient number (n) | Complete response | Partial response | Stable disease | Median overall survival (OS)/ progression free survival (PFS)/time to progression (TTP) |
|---|---|---|---|---|---|---|
| Tanabe et al., 2013 | CVD chemotherapy | N=17 | 0% | 47.1%1 | 23.5% | PFS responders 40 months |
| Huang et al., 2008 | CVD chemotherapy* | N=18 (n=8 with SDHB orSDHDmutation) | 11% | 44% | OS responders/non-responders 3.8/1.8 years | |
| Averbuch et al., 1988 | CVD chemotherapy* | N=14 | 57% (complete plus partial response) | PFS 21 months (7 to >34 months) | ||
| Jawed et al., 2018 | Prolonged CVD chemotherapy (median 20,5 cycles) | N=12 (all with SDHBmutations) | 16,7% (2/12) | 66,7% (8/12) | 0%2 | OS/PFS 3.3/2.6 years |
| Ayala-Ramirez et al., 2012 | different chemotherapy regimens | N=54 (n=52 evaluable) (all progressive disease at baseline) | 33%3 | OS responders/non-responders 6.4/3.7 years | ||
| Hadoux et al. | Temozolomide monotherapy | N=15 (n=10 with SDHBmutations) | 0% | 33%4 | 47%4 | PFS 13.3 months |
| Nastos et al., 2017 | [131I]-MIBG vs. [177Lu]/ [90Y]DOTATATE(PRRT) | N=22 Patients (n=11 MIBG, n=9 [177Lu]DOTATATE, n=2 combinations, n=15 PGL, n=7 PCC) (all progressive disease at baseline) | MIBG: 63% vs. [177Lu]DOTATATE100% | OS/PFS MIBG 41.2/20.6 months vs. OS/PFS [177Lu]DOTATATE 60.8/38.5 months Sub-group PGLs: OS/PFS MIBG 22.8/14.4 months vs. OS/PFS [177Lu]DOTATATE 60.8/38.5 months (p<0,05) |
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| Van Essen et al., 2006 | [177Lu] DOTATATE | N=12 (n=1 PCC, n=5 HN, n=6 other PGLs) | 0% | 16.7%5 | 50%5 | TTP 11 and 5 months, respectively in 2 patients, median TTP to progression not reached in PGL patients |
| Forrer et al., 2008 | [90Y] DOTATOC, 3 combined with [177Lu] DOTATATE | N=28 (n=9 PCC, n=19 PGL) (all progressive disease at baseline) | 0% | 25%1 | 46.4% | TTP 3 to > 42 months, median TTP 18±14 (6-44) months |
| Kong et al., 2017 | [177Lu] DOTATATE, 9 combined with radiosensitizing chemotherapy | N=20 (n=8 PCC, n=5 HN PGLs, n=5 abd. PGLs, n=2 HN plus abd. PGLs) (n=7 SDHB, n=1 SDHD, n=2 no mutation, n=10 unknown) | 0% | 36%1 | 50% | PFS 39 months, OS not reached |
| Puranik et al., 2015 | [90Y] DOTATOC n=4 combined with 177[Lu]-DOTATATE* | N=9 HN PGLs | 0% | 0% | 100% | - |
| Yadav et al., 2019 | 177[Lu]-DOTATATE | N=25 PGLs | 0% | 56% 28% | 28% 56% | PFS 32 months, OS not reached |
| Imhof et al. 2011 | [90Y] DOTATOC (phase II, prospective) | N=39 (n=11 PCCs, n=28 PGLs) (all progressive disease at baseline) | ns | 47% 18% | ns | Mean OS in PCC/PGL 32/82 months |
| Zandee et al., 2019 | 177[Lu]-DOTATATE | N=30 (n=17 parasympathetic PGLs, n=10 sympathetic PGLs, n=3 PCCs) | 0% | 23% | 67% | **PFS in PGL#/PCC 13/10 months, respectively |
| Satapathy et al., 2019 | 177[Lu]/[90Y] -DOTATATE, [90Y] DOTATOC | Meta-analysis from 12 studies: n= 201 | 0% | 25%6 | 59%6 | - |
| Taieb et al., 2019 | [90Y]/ 177[Lu]-DOTATATE | Meta-analysis n= 179 (n=234) | 90% (partial response plus stable disease) | |||
| Ayala-Ramirez et al., 2012 | TKI sunitinib(retrospective) | N=17 (n=14evaluable) | 0% | 21%(3/14) | 36%(5/14) | PFS 4.1 months (75% (6/8)62.5% (5/8)with stable disease or partial responseSDHBmutation carriers) |
| Jasim et al., 2017 | TKI pazopanib* | N=7 (6 evaluable) | 17% | PFS/OS 6.5/14.8 months | ||
| Oh et al., 2012 | MTORC1 inhibitor everolimus (phase II, prospective) | N=7 | 0% | 0% | 71% (5/7) |
Not assessed
PFS 3.8 months |
Added to caption: Minor response: any shrinkage of tumor which does not fulfill the criteria of partial response. If not indicated otherwise, reported minor/minimal response is included in “Stable disease.” The following footnotes were added:
1 Minor/minimal response included
2 Minor/minimal response excluded (2/12)
3 Overall response rate
4 According to RECIST plus PERCIST
5 Including all 12 patients of which only 11 were evaluable
6 This meta-analysis provides an overall response rate of 25% (n=179) and a disease control rate of 84% (n=151)
* prospective
**Overall PFS 30 months, parasympathetic PGL 91 months
# sympathetic