| | Study | Year | Tumor/patient | Localisation | Symptoms | Therapy | Follow-up | IHC/Mol |
| | Hunsuck [53] | 1968 | ES/m 33 y | Maxilla | Swelling | () RTX 3000 rads | 18 mts: DF | n.s. | | () Subtotal maxillectomy |
| | Roca et al. [54] REV. | 1968 | ES m/3 y | Maxilla | Proptosis | RTX 4000 r + resection | 11 y: DF | n.s. | | ES m/7 y | Maxilla | Swelling | Curettage, RTX 5000 r + C | 5 y: DF | n.s. |
| | Brownson and Cook [55] | 1969 | ES f/6 y | Maxilla | Facial swelling | () RTX (n.f.s.) | n.s. | n.s. | | () VC | n.s. |
| | Fernandez et al. [56] REV. | 1974 | ES/2 Pat. | Maxilla | n.s. | Chemoradiation (n.f.s.) | n.s. | n.s. |
| | Ferlito [57] REV. | 1978 | ES/m, 29 y | Maxilla | Congestion | RTX + Adr, V, Bl | 10 mts: died | n.s. | | ES/m, 44 y | Maxilla | Swelling | Resection, RTX, Adr, V, CCNU | 8 mts: DF | n.s. | | ES/m, 43 y | Maxilla | Epistaxis | RTX + Adr, V, CCNU | 1 y: died | n.s. | | ES/m, 44 y | Maxilla | Epistaxis | RTX + Adr, V, CCNU | 3 mts: DF | n.s. |
| | Komray [58] | 1979 | ES/m, 15 y | Max sin | Epistaxis, exopht. | () RTX 6000 rads | 1 y: DF | n.s. | | () VAdC |
| | Strong et al. [52] REV. | 1979 | ES/m, 3 y | Maxilla | n.s. | AMC + RTX 4000 rads | 23 y: DF | n.s. | | ES/m, 7 y | Maxilla | n.s. | C + RTX 6500 rads | 14 y: died | n.s. | | ES/m, 9 y | Maxilla | n.s. | CVAD + RTX 6000 rads | 4 y: DF | n.s. |
| | Pontius and Sebek [59] | 1981 | ES/m, 39 y | Max. sin. | Obstruct., lacrimat. | () Resection | 2 y: DF | n.s. | | () RTX 7500 rads |
| | Hossfeld et al. [60] | 1982 | ES/n.s. | Maxilla | n.s. | Chemoradiation (n.f.s.) | n.s. | n.s. |
| | Slootweg et al. [61] | 1983 | PNET/m, 10 y | Maxilla | Swelling | () Subtotal maxillectomy | 3 y: DF | n.s. | | () RTX 50 Gy |
| | Bacchini et al. [62] | 1986 | ES/f, 7 y | Maxilla | n.s. | () Curettage, RTX 6000 rads | 5 y 6 mts: DF | n.s. | | () Adj. CVAD |
| | Siegal et al. [42] REV. | 1987 | ES/m, 14 y | Maxilla | Swelling | VCD/VACD + RTX 4300 rads | 10 y: DF | n.s. | | ES/m, 9 y | Maxilla | Swelling | VCD/VACD + RTX 6000 rads | 8 y: DF | n.s. | | ES/f, 8 y | Maxilla | Swelling | VCD/VACD + RTX 6550 rads | 7 y 5 mts: DF | n.s. | | ES/f, 15 y | Maxilla | Swelling | VCD/VACD + RTX 5500 rads | 11 mts: DF | n.s. |
| | Amin et al. [63] | 1990 | ES/f, 10 y | Maxilla | Obstruct., swelling | () Lateral rhinotomy | n.s. | n.s. | | () RTX, not performed |
| | Yeo et al. [64] | 1991 | PNET/f, 7 y | Maxilla | Swelling, pain | () Neoadj. VAC | 9 y: DF | n.s. | | (2) Partial maxillectomy | | () Adj. C + RTX 50 Gy |
| | Filiatrault et al. [65] | 1993 | PNET/f, 11 y | Max sin | Obstruct. | Chemoradiation (n.f.s.) | 10 mts: SD | n.s. |
| | Jones and McGill [66] REV. | 1995 | PNET/f, 13 y | Max sin | n.s. | Chemoradiation (n.f.s.) | 2 y: DF | n.s. |
| | Shah et al. [67] | 1995 | PNET/m, 42 y | Max sin | Swelling | () CAVCisPt, I, VP-16, E | 9 mts: died | n.s. | | () RTX (n.f.s.) |
| | Ibarburen et al. [68] | 1996 | PNET/f, 20 mts | Maxilla | Swelling | n.s. | 3 y: DF | n.s. |
| | Fiorillo et al. [69] | 1996 | ES/m, 7 y | Maxilla | Swelling | CVAIE + RTX 6000 cGy | 52 mts: DF | n.s. | | ES/f, 22 y | Maxilla | Swelling | CVAIE + RTX 6000 cGy | 41 mts: DF | n.s. |
| | Zheng et al. [70] | 1998 | ES/m, 16 y | Maxilla | Swelling | n.s. | n.s. | n.s. |
| | Allam et al. [71] REV. | 1999 | ES/9 Pat. | Maxilla | n.s. | () RTX + VAdC + IEP/VAIA | 3.4 y (mean) | n.s. | | () Surgery, n.s. |
| | Toda et al. [72] | 1999 | PNET/f, 57 y | Max. sin. | Obstruct. | () VAdC | 3 mts: died | MIC2− | | () RTX 40 Gy |
| | Daw et al. [51] | 2000 | ES | Maxilla | n.s. | () Resection, RTX 3500 cGy | 16 y 9 mts: DF | n.s. | | () Adj. CVAD, Carm |
| | Wexler et al. [73] | 2003 | ES | Max sin | Congestion, swelling | () Neoadj. VAdC + IE | 3 y: DF | MIC2+, EWS-FLI1 | | () Subtotal maxillectomy | | () Adjuv. chemotherapy (n.f.s.) |
| | Kao et al. [74] | 2002 | PNET/m, 74 y | Maxilla | Swelling | CisPtEI | 4 mts: died | CD99+ |
| | Alobid et al. [75] | 2003 | PNET/f, 23 y | Max. sin. | Obstruct. | () Lateral rhinotomy | 59 mts: DF | CD99+ | | () Adj. CVAD + RTX 60 Gy |
| | Harman et al. [76] | 2003 | ES/f, 40 y | Max sin | Obstruct. | () VAC | n.s. | CD99+ | | () RTX 2000 cGy |
| | Windfuhr [44] REV. | 2004 | PNET/m, 7 y | Max. sin. | Exopht., vision loss | () Lateral rhinotomy | 17 mts: DF | n.s. | | () Adj. VIDE/VACD | | () RTX 45 Gy |
| | Howarth et al. [43] | 2004 | ES/m, 9 y | Maxilla | Swelling | () VIDE (inoperable) | n.s. | CD99+ | | () RTX (n.f.s.) |
| | Sun et al. [77] | 2007 | PNET/f, 49 y | Maxilla | Swelling | () Total right maxillectomy | 3 mts: DF | CD99+, EWS-FLI1 | | () Radiotherapy 60 Gy |
| | Infante-Cossio et al. [78] | 2005 | ES/m, 17 y | Max. sin. | Swelling | () Neoadj. VCAIE | 8 y: DF | CD99+ | | () Complete resection | | () Adj. chemotherapy (n.f.s.) |
| | Coskun et al. [79] | 2005 | ES/f, 16 y | Max sin | Swelling | () Surgery declined | 1 y: DF | CD99+ | | () RTX + CVAD/IE |
| | Varshney et al. [80] | 2007 | ES | Maxilla | Swelling | () Resection | n.s. | CD99+ | | () Adj. VAC + RTX 2000 cGy |
| | Thariat et al. [81] | 2008 | ES/m, 26 y | Max. sin. | Swelling | () Neoadj. CD | 11 y: local recurrence | t(11;22) EWSR1 | | () Surgery | | () Adj. AV |
| | Prasad et al. [82] | 2008 | ES/m, 18 y | Maxilla | Swelling | () Extended maxillectomy | 1 y: DF | n.s. | | () Adj. chemotherapy (n.f.s.) |
| | Mohindra et al. [83] | 2008 | PNET/m, 5 y | Max. sin. | Swelling, exopht. | () VIDECAdr | n.s. | CD99+, EWS-FLI1 | | () RTX 55.8 Gy |
| | Bornstein et al. [84] | 2008 | ES/f, 19 y | Max. sin. | Pain in teeth | () neoadj. VIDE (EU-Ew99) | n.s. | CD99+, EWS-FLI1 | | () Hemimaxillectomy | | () Adj. VAC + RTX 46 Gy |
| | Kawabata et al. [85] REV. | 2008 | ES, PNET/m, 12 y | Max sin | Swelling | () VDCIE | 20 mts: DF | CD99+, EWS-ERG | | () RTX 45 Gy |
| | Ataergin et al. [86] | 2009 | ES/n.s. | Maxilla | n.s. | () Neoadj. IEAM | died | n.s. | | () Resection | | () Adj. RTX (n.f.s.), VD IE AC |
| | Piloni et al. [87] | 2009 | ES/2 Pat. | n.s. | n.s. | n.s. | n.s. | n.s. |
| | Gupta et al. [88] | 2009 | ES/m, 30 y | Maxilla | Swelling, congestion | () Excision | n.s. | CD99+ | | () Adj. chemoradiation (n.f.s.) |
| | Hormozi et al. [89] | 2010 | PNET/f, 28 y | Maxilla | Diplopia | () Maxillectomy | 6 mts: DF | CD99+ | | Metastasis | () γ-knife (optic chiasma) | | Parasellar | () Adj. VAI + RTX 80 Gy |
| | Dadhe et al. [90] | 2010 | ES/m, 12 y | Max. sin. | Swelling, pain | () Extended maxillectomy | 1 y: DF | n.s. | | () Adj. chemotherapy (n.f.s.) |
| | Davido et al. [91] | 2011 | ES/m, 25 y | Max. sin. | Swelling, pain | () Neoadj. IDCisPt | 5 y: DF | CD99+ | | () Part maxillectomy | | () Adj. IDCisPt |
| | Hafezi et al. [92] REV. | 2011 | ES/f, 69 y | Max. sin. | Obstruct., epistaxis | Chemoradiation (n.f.s.) | 6 mts: DF | CD99+, EWS-FLI1 | | ES/f, 45 y | Max. sin. | Obstruct., epistaxis | Chemoradiation (n.f.s.) | 14 mts: died | CD99+, EWS-FLI1 | | ES/m, 54 y | Maxilla | Obstruct., epistaxis | Chemoradiation (n.f.s.) | 17 mts: died | CD99+ EWSR1 | | ES/m, 25 y | Max. sin. | Obstruct., epistaxis | Surgery (n.f.s.) | 26 mts: DF | CD99+ EWSR1 | | ES/f, 13 y | Max. sin. | Obstruct., epistaxis | Surgery (n.f.s.) | 4 mts: DF | CD99+ EWSR1 | | ES/f, 15 y | Max. sin. | Obstruct., epistaxis | Chemoradiation (n.f.s.) | 14 y: DF | CD99+ EWSR1 |
| | Yeshvanth et al. [93] | 2012 | ES/f, 29 y | Maxilla | Obstruct., epistaxis | () Neoadj. VACDex | 1 y: DF | CD99+ | | () Resection |
| | Kaler and Sheriff [94] | 2013 | ES/m, 22 y | Maxilla & orbit | Swelling, pain | () Craniofacial resection | 2 y: local recurrence | CD99+ | | () Chemoradiation (n.f.s.) |
| | Biswas et al. [49] | 2014 | ES/14 Pat. | Maxilla (sin.) | n.s. | () VDCIE | 58 mts (median) | CD99+ | | () RTX or surgery ± RTX 50–60 Gy | | () Adj. chemotherapy (n.f.s.) |
| | Grevener et al. [50] | 2016 | ES/7 Pat. | Maxilla | n.s. | () VIDE | 3.3 y (median) | n.s. | | () RTX or surgery ± RTX 44–54 Gy |
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