Table 2.
Comprehensive literature review of adjuvant and metastatic treatments and clinical outcomes for published GNET case reports/case series.
| Reference | Case No. | Age/Gender | Local/Recurrent/Metastatic Disease | Adjuvant/Metastatic Treatment (All Patients Underwent Primary Tumor Resection) |
Clinical Outcome |
|---|---|---|---|---|---|
| Alyousef et al., 2017 [9] | 1 | 18 M | Local recurrent | No | DFS 36 mos, DD 12 mos after local recurrence |
| Antonescu et al., 2006 [10] | 3 | 81 F | De novo metastatic | Metastatic: Liver metastectomy and peritoneal implants removal (NA) | NA |
| 42 F | Local | NA | NA | ||
| 42 F | De novo metastatic | NA | NA | ||
| Boland et al., 2016 [44] | 1 | 46 F | Local | NA | NA |
| Chang et al., 2020 [1] | 19 | 29 F | De novo metastatic | NA | NA |
| 44 M | De novo metastatic | No | OS 61 mos | ||
| 41 M | Recurrent metastatic | Adjuvant: no Metastatic: radiofrequency ablation of liver metastasis (CR), no systemic treatment |
DFS 8 mos NED F/U 63 mos |
||
| 42 M | Local | Adjuvant: dacarbazine + cisplatin | NED F/U 46 mos | ||
| 51 F | Recurrent metastatic | NA | DFS 11 mos OS 36 mos |
||
| 30 F | Recurrent metastatic | Adjuvant: no Metastatic: 1st line: sunitinib (stopped due to SE), 2nd line: anlotinib (PR for 6 mos) |
DFS 14 mos AD F/U 43 mos |
||
| 48 F | NA | NA | NA | ||
| 42 F | Local | No | NED F/U 3 mos | ||
| 41 F | Recurrent metastatic | NA | DFS 4 mos AD F/U NA |
||
| 56 F | De novo metastatic | Metastatic: Liver metastectomy, no systemic treatment | NED F/U 33 mos | ||
| 30 F | NA | NA | NA | ||
| 59 M | Local | No | NED F/U 29 mos | ||
| 27 F | Recurrent Metastatic | Adjuvant: no Metastatic: apatinib (PR for 4 mos stopped due to SE) |
DFS 13 mos AD F/U 26 mos |
||
| 47 M | Recurrent metastatic | Adjuvant: no Metastatic: 1st line: pazopanib + pembrolizumab (stopped due to SE), 2nd line: lenvatinib + pembrolizumab (PD), 3rd line: epirubicin + ifosfamide (SD for 3 mos) |
DFS 27 mos AD F/U 48 mos |
||
| 36 M | Local | Adjuvant: Vincristine amide + Adriamycin + cyclophosphamide | NED F/U 3 mos | ||
| 40 F | Local | Adjuvant: Adriamycin + dacarbazine | NED F/U 8 mos | ||
| 41 F | Recurrent Metastatic | Adjuvant: no Metastatic: 1st line: oxaliplatin (PD), 2nd line: irinotecan (PD), 3rd line: paclitaxel (PD), apatinib (SD for 5 mos) |
DFS 17 mos AD F/U 29 mos |
||
| 64 M | Local | Adjuvant: adriamycin + ifosfomaide | NED F/U 14 mos | ||
| 55 M | NA | NA | NA | ||
| Comunoglu et al. 2015 [45] | 1 | 9 M | Local | Adjuvant: chemotherapy (NA) | NED F/U 12 mos |
| Damle et al., 2021 [8] | 1 | 56 M | De Novo Metastatic | Metastatic: VAC/IE (PR after 3 cycles then PR/SD additional 11 cycles) | AD F/U 3 mos |
| Friedrichs et al., 2005 [39] | 1 | 41 M | Recurrent Metastatic | Adjuvant: no Metastatic: ifosfamide, vincristine, actinomycin D, followed by ifosfamide and epirubicin (NA) |
DFS: 6 mos AD F/U NA |
| Gadde et al., 2021 [13] | 1 | 36 F | De novo metastatic | Metastatic: liver metastectomy, dacarbazine + gemcitabine (PD) | OS 4 mos |
| Harshavardhini et al., 2021 [14] | 1 | 33 M | Local | Adjuvant: vincristine, etoposide, adriamycin, cyclophosphamide, mesna and ifosfamide | NED F/U 8 mos |
| Huang et al., 2019 [15] | 1 | 30 F | Local | Adjuvant: ifosfamide + epirubicin | NED F/U 6 mos |
| Huang et al. 2020 [16] | 4 | 45 F | Local | No | NED F/U 41 mos |
| 34 F | Local | No | NED F/U 17 mos | ||
| 81 M | Local with residual disease | No | AD F/U 8 mos | ||
| 68 M | De novo metastatic | Metastatic: chemotherapy (PD) | OS 5 mos | ||
| Insabato et al., 2015 [46] | 1 | 29 M | Recurrent metastatic | Adjuvant: no Metastatic: 1st line: IE (PR/SD for 8 mos) 2nd line: ifosfamide alone (SD) |
DFS 36 mos AD F/U 39 mos |
| Kansal & Rao, 2017 [17] | 1 | 55 F | NA | NA | NA |
| Keditsu et al., 2017 [18] | 1 | 37 F | De novo metastatic | Metastatic: liver metastectomy followed by pseudoadjuvant VAC/IE | NED F/U 36 mos |
| Kim et al. [47] | 1 | 21 M | Local | Adjuvant: RT | NED F/U 5 mos |
| Kong et al., 2014 [19] | 1 | 17 M | NA | NA | NA |
| Li et al., 2020 [48] | 2 | 17 M | Local | No | NED F/U 10 mos |
| 62 M | De novo metastatic | No | AD F/U 6 mos | ||
| Libertini et al., 2018 [34] | 6 | 59 F | Recurrent metastatic | Adjuvant: no Metastatic: dacarbazine (PD) |
DFS 11 mos OS 18 mos |
| 28 F | Local recurrent | Resection for local recurrence, no systemic treatment | DFS 109 mos NED F/U 161 mos |
||
| 27 F | Recurrent metastatic | NA | DFS 2 mos OS 4 mos |
||
| 33 M | Recurrent metastatic | Adjuvant: no Metastatic: no |
DFS 2 mos OS 8 mos |
||
| 48 M | Recurrent local/metastatic | NA | DD F/U NA | ||
| 27 M | De novo metastatic | Metastatic: metastectomy (peritoneal resection), no systemic treatment | NED F/U 2 mos | ||
| Lyle et al., 2008 [42] | 7 | 46 M | Local | Adjuvant: chemotherapy (NA) | NED F/U 7 mos |
| 62 M | De novo metastatic | Metastatic: chemotherapy (NA) | OS 12 mos | ||
| 49 M | De novo metastatic | No | OS 2 mos | ||
| 60 F | De novo metastatic | NA | NA | ||
| 29 F | De novo metastatic | NA | NA | ||
| 60 M | De novo metastatic | Metastatic: chemotherapy (NA) | OS 28 mos | ||
| 55 F | NA | NA | NA | ||
| Okada et al., 2020 [29] | 1 | 38 F | De novo metastatic | Metastatic: liver Metastectomy, no systemic treatment | NED F/U 36 mos |
| Shah et al., 2015 [28] | 1 | 28 F | Recurrent metastatic | Adjuvant: RT, no systemic treatment Metastatic: IL-2 (PD), anti-CTLA4 (SD for 10 mos), anti-PD-L1 (SD for 7 mos), IL-15 (PD) |
DFS 48 mos AD F/U 72 mos |
| Singh et al., 2020 [22] | 1 | 61 M | Recurrent metastatic | Adjuvant: cisplatin+etoposide Metastatic: 1st line: capecitabine+temozolomide (PD), 2nd line: everolimus (SD for 5 mos), 3rd line: pazopanib (PD), 4th line: sunitinib (SD for 3 mos) |
DFS 84 mos OS 13–15 mos |
| Sivasubramaniam et al., 2021 [32] | 1 | 46 F | Recurrent metastatic | Adjuvant: no Metastatic: metastectomy (intraabdominal lymph nodes) followed by pseudoadjuvant pazopanib |
DFS 17 mos NED F/U NA |
| Stockman et al., 2012 [3] | 16 | 30 F | NA | NA | AD F/U 21 mos |
| 35 M | NA | NA | OS 18 mos | ||
| 33 M | NA | NA | AD F/U 1.5 mos | ||
| 50 F | NA | NA | AD F/U 24 mos | ||
| 20 F | NA | NA | NED F/U 20 mos | ||
| 52 M | NA | NA | OS 22 mos | ||
| 46 M | NA | NA | NA | ||
| 34 F | NA | NA | OS 19 mos | ||
| 37 F | NA | NA | NA | ||
| 77 F | NA | NA | OS 106 mos | ||
| 31 M | NA | NA | OS 3 mos | ||
| 17 M | NA | NA | NA | ||
| 30 M | NA | NA | AD F/U 36 mos | ||
| 60 F | NA | NA | NED F/U 41 mos | ||
| 56 M | NA | NA | NA | ||
| 28 F | NA | NA | OS 23 mos | ||
| Song et al., 2018 [31] | 1 | 23 M | Local recurrence | Adjuvant: RT Local recurrence: surgical resection, no systemic treatment |
DFS 12 mos NED F/U 24 mos |
| Subbiah et al., 2016 [49] | 1 | 27 F | De novo metastatic | Metastatic: metastectomy (liver and others), cryotherapy, palliative RT, crizotinib + pazopanib (PR for 1.5 yrs) | AD F/U 2.8yrs |
| Wang et al., 2020 [50] | 1 | 30 F | De novo metastatic | Metastatic: chemotherapy (NA, PR) | AD F/U 6 mos |
| Washimi et al., 2017 [40] | 1 | 32 F | Recurrent metastatic | Adjuvant: ifosfamide + adriamycin Metastatic: NA |
DFS 38 mos AD F/U NA |
| Wolak et al., 2018 [33] | 1 | 12 M | Recurrent metastatic |
Adjuvant: vincristine + adriamycin + ifosfamide + dactinomycin, followed by carboplatin + epirubicin + vincristine + actinomycin D + ifosfamide + etoposide Metastatic: thermal ablation (PD), liver metastectomy (PD), 1st line: carboplatin, epirubicin, vincristine, actinomycin D, ifosfamide and etoposide (PD), 2nd line: pazopanib (PD) |
DFS 8 weeks OS 18 mos |
| Yagi et al., 2020 [43] | 1 | 66 F | De novo metastatic | Metastatic: 1st line: dabrafenib + trametinib (PR for 3 mos), 2nd line: nivolumab + ipilimumab (PD) | OS 21 mos |
| Yegen et al., 2015 [41] | 1 | 25 F | De novo metastatic | Metastatic: Liver Metastectomy followed by chemotherapy (NA, PD) | AD F/U 47 mos |
| Zambrano et al., 2003 [2] | 6 | 15 F | De novo metastatic | Metastatic: chemotherapy (NA, PD) | OS 16 mos |
| 21 F | De novo metastatic | Metastatic chemotherapy (NA, PD) | OS 12 mos | ||
| 35 F | Recurrent metastatic | Adjuvant: No Metastatic: NA |
DFS 12 mos AD F/U NA |
||
| 37 F | Local | NA | NA | ||
| 13 M | Recurrent local/metastatic | Adjuvant: chemotherapy (NA) Recurrence: total gastrectomy for local recurrence followed by pseudoadjuvant chemotherapy (NA) |
DFS 7 mos AD F/U 5 mos |
||
| 32 M | Local | NA | NA | ||
| Zhan et al., 2019 [51] | 1 | 33 F | Recurrent metastatic | Adjuvant: chemotherapy (NA) Metastatic: metastectomy (mesentery), sunitinib (SD for 12 mos) |
DFS 14 mos AD F/U 12 mos |
| Zhao et al., 2014 [25] | 1 | 33 F | Local | Adjuvant: ifosfamide + epirubicin | NED F/U NA |
| Zhao et al., 2017 [24] | 2 | 57 M | local | No | NED F/U 16 mos |
| 24 M | De novo metastatic | Metastatic: 1st line: paclitaxel + gemcitabine (PD), 2nd line: vinorelbine + gemcitabine (PD), 3rd line: apatinib (SD for 2 mos), 4th line: apatinib+temozolomide (SD for 3–4 mos) | AD F/U 55 mos |
DFS = Disease Free Survival, CR = Complete Response, PR = Partial Response, SD = Stable Disease, DD = Died of Disease, NED = No Evidence of Disease, NA = Not Available, OS = Overall Survival, AD = Alive with Disease, F/U = Follow Up, SE = side effects, mos = months, yrs = years, RT = Radiation Treatment, VAC/IE = vincristine + adriamycin (actinomycin D) + cyclophosphamide/ifosfamide + epirubicin, IL = Interleukin, CTLA4 = T-lymphocyte-associated protein 4, PD1 = anti-programmed cell death protein 1.