Table 1 Characteristics of studies reporting survival data for nfMPNSTs and sMPNSTs
First author | Published year | Country | Total no. of patients (analyzed) | No. of patients in NF1 arm/sporadic arm | Events | No. of gender female/male | Mean age in years (SD) | Median follow-up in months (range) |
|---|---|---|---|---|---|---|---|---|
Angelov | 1998 | Toronto, Canada | 18 | 7/11 | 7 | 10/8 | 45.9 (21.4) | 67 (9–81) |
Ariel | 1993 | New York, USA | 74 | 30/44 | 48 | Â | Â | Â |
Arpornchayanon | 1984 | Tokyo, Japan | 16 | 2/14 | 5 | 8/8 | 40.94 (9.3) | (7–144) |
Bergamaschi | 2017 | Italy | 73 | 35/38 | 60 | 36/37 |  ≤ 10 years: 27 (37%)  > 10 years: 46 (63%) | 180 (42–430) |
Bojsen-Moller | 1984 | Denmark | 29 | 8/21 | 20 | 13/16 | 40.34 (19.43) | 60 (24–240) |
Brekke | 2009–2010 | Norway, Sweden, Netherlands | 82 | 37/45 | 57 | 38/44 | 40.6 (20.7) | 42 (1–369) |
Carli | 2005 | Italy, Germany | 167 | 29/138 | 89 | 84/83 | 10.5 (5.83) | 87.6 (28.5–346.8) |
Casanova | 1999 | Italy | 24 | 7/17 | 16 | 11/13 | 10.06 (4.99) | 36 (6–250) |
Cashen | 2004 | USA | 80 | 18/62 | 15 | 41/39 | 36 (17) | 19.7 (8–276) |
D’Agostino | 1963 | USA | 41 | 19/22 | 30 | 21/20 | 37.8 (14.9) | 21 (3–192) |
Daimaru | 1985 | Japan | 28 | 11/17 | 18 | 19/9 | 40.25 (18.59) | 36 (3––204) |
De Cou | 1995 | USA | 27 | 11/16 | 15 | 14/13 | 13.89 (4.57) | 25 (3–180) |
De Vasconcelos | 2017 | Brazil | 92 | 41/51 | 53 | 51/41 | 43.5 (23.77) | 24.8 (2–252) for NF1, 46.5 (3–208) for sporadica |
Demir | 2012 | Turkey | 13 | 4/9 | 8 | 6/7 | 10.2 (4.79) | 8.5 (1–130) |
Doorn | 1995 | Netherlands | 22 | 11/11 | 16 | 8/14 | 39.5(22.99) | 24 (1–153) |
Ducatman | 1986 | USA | 120 | 62/58 | 88 | 68/52 | 34 (20.29) | 25.8 (0.11–120) |
Evans | 2002 | UK | 61 | 24/37 | 51 |  | 49.44 (20.65) | 34.6 (11.1–204) |
Fan | 2013 | China | 146 | 17/129 | 53 | 67/79 |  < 40 years: 71 (49%)  ≥ 40 years: 75 (51%) |  |
Ganju | 2001 | USA | 12 | 5/7 | 7 | 3/9 | 40.9(19.6) | 54 (6–119) |
Guellec | 2016 | France | 106 | 68/38 | 49 | 41/65 | 45.25 (24.2) | 66 (39–90) |
Halling | 1996 | USA | 28 | 14/14 | 22 |  | 39.2 (17.4) | 26 (1.5–251) |
Hagel | 2007 | Germany | 52 | 38/14 | 34 | 24/28 | 36.3 (17.1) | Â |
Holtkamp | 2007 | Germany | 35 | 22/13 | 20 | 17/18 | 39.4 (19.6) | 29 (3–200) |
Hong | 2017 | Korea | 11 | 8/3 | 2 | 4/7 | 9.5 (6.04) | 136.6 (2.3–167.1) |
Hruban | 1990 | USA | 43 | 23/20 | 30 | 22/21 | 40 (16.7) | 31 (2–324) |
Hwang | 2017 | Korea | 95 | 33/62 | 57 | 45/50 | 42.38 (21.18) | 166.76 (5.94–329.53) |
Kamran | 2012 | USA | 84 | 24/60 | 39 | 37/47 | 43.2 (22.7) | 19 (0–329) |
Kar | 2006 | India | 24 | 5/19 | 10 | 5/19 | 44.3 (18.8) | 38a |
Kourea | 1998 | USA | 25 | 15/10 | 20 | 12/13 | 31.6 (12.2) | 11 (2–221) |
Kunisada | 1997 | Japan | 11 | 5/6 | 9 | 4/7 | 44.5 (17.57) | 15 (2–52) |
Lamm | 2013 | Austria | 14 | 6/8 | 10 | 7/7 | 50.25 (18.72) | 41 (1–108) |
Lodding | 1986 | Sweden | 14 | 1/13 | 10 | 9/5 | 42.14 (17.6) | 24 (2–216) |
Longhi | 2010 | Italy | 62 | 22/40 | 40 | 23/39 | 44 (18.5) | 54 (12–194) |
Loree | 2000 | USA | 17 | 7/10 | 10 | 8/9 | 41.25 (22.4) | 139.2 (40.8–432) |
Martin | 2020 | USA | 70 | 26/44 | 25 | 32/38 | All ≤ 18 years | (1.4–150) |
McCarron | 1998 | USA | 15 | 12/3 | 7 | 7/8 | 38(21.03) | 16 (3–120) |
McCaughan | 2007 | Scotland | 25 | 12/13 | 17 |  | 59 (21.2) | 31.12 (0.9–139.55) |
Meis | 1992 | USA | 57 | 13/44 | 28 | 26/31 |  < 7 years: 23 (40%)  ≥ 7 yeas: 34 (60%) | 22 (2–230) |
Mertens | 2000 | Sweden | 19 | 7/12 | 12 | 9/10 | 34.53 (16) | 36 (7–84) |
Miao | 2019 | USA | 259 | 77/182 | 223 | 132/127 | 40.5 (27.5) | 75.87 (0.28–378.22) |
Okada | 2007 | Japan | 56 | 25/31 | 32 | 34/22 | 45 (20.57) | 44.6 (2–156) |
Owosho | 2018 | USA | 13 | 9/4 | 8 | 6/7 | 30.8 (16) | 24 (13–141) |
Porter | 2009 | UK | 123 | 33/90 | 56 |  | Median: NF1 26, sporadic 53 | (6–252) |
Raney | 1987 | USA | 24 | 16/8 | 14 | 14/10 | 10.56 (5.53) | 17 (4–291.6) |
Scheithauer | 2009 | USA | 12 | 4/8 | 11 | 2/10 | 41.5 (15.2) | 5 (1.5–180) |
Schmidt | 1999–2000 | Germany | 26 | 6/20 | 13 | 14/12 | 48.27 (16.8) | 27 (2–168) |
Sordillo | 1981 | USA | 165 | 65/100 | 104 | 89/76 | 41.4 (26.3) | Â |
Tabone-Eglinger | 2008 | France | 52 | 26/26 | 23 | 22/30 | 23 (15) | Â |
Trojanowski | 1980 | USA | 18 | 2/16 | 8 | 13/5 | 48.3 (19) | 54 (1–90) |
Valentin | 2016 | France | 294 | 106/188 | 182 |  |  | 87.6 (2.08–279.1) |
Van Noesel | 2019 | Netherlands | 51 | 25/26 | 20 | 26/25 | 12.2 (6.1) | 64.6 (1.3–147.7) |
Wang | 2013 | China | 13 | 3/10 | 2 | 5/8 | 57.2 (16.8) | (4–132) |
Wang | 2015 | China | 43 | 6/37 | 22 | 18/25 |  < 50 years: 23 (53%)  ≥ 50 years: 20 (47%) | 24 (2–115) |
Watson | 2004 | USA | 40 | 24/16 | 26 | 23/17 | 38.48 (15.17) | 18 (2–126) |
White | 1971 | USA | 15 | 10/5 | 9 | 7/8 | 36.87 (11.59) | 16 (2–156) |
Wong | 1998 | USA | 134 | 32/102 | 69 | 52/82 | 41.75 (22.03) | 53 (7–280) |
Yu | 2011 | USA | 122 | 47/75 | 88 | 67/55 | 38.25 (18.98) | 18 (1–180) |
Yuan | 2017 | China | 159 | 70/89 | 101 | 78/81 | 40.25 (20.69) | 31 (2–199) |
Zhou | 2016 | USA/China | 51 | 35/16 | 23 | 22/29 |  ≥ 40 years: 22 (43%)  < 40 years: 29 (57%) |  |