Twenty-Year OS, PFS, EEFS, and EMS at Last Followup With Crude HRs (95% CI) and Unadjusted Regression Coefficients β, Respectively
Independent Variable . | OS (95% CI) . | PFS (95% CI) . | EEFS (95% CI) . | EMS (95% CI) . | |||||||
---|---|---|---|---|---|---|---|---|---|---|---|
20-Year OS . | Crude HR . | P . | 20-Year PFS . | Crude HR . | P . | 20-Year EEFS . | Crude HR . | P . | Crude β . | P . | |
Age | |||||||||||
>5 y | 88.7 (76.1–100.0) | 1.00 | 53.4 (36.1–70.8) | 1.00 | 23.8 (13.2–34.5) | 1.00 | −0.02 (−0.07–0.04) | .600 | |||
1–5 y | 80.0 (60.6–99.3) | 1.05 (0.28–3.94) | .945 | 49.5 (34.4–64.5) | 1.17 (0.69–2.00) | .566 | 25.6 (11.1–40.0) | 0.64 (0.43–0.97) | .034 | ||
<1 y | 62.9 (32.6–93.1) | 5.29 (1.32–21.22) | .019 | 6.7 (0.0–23.2) | 5.26 (2.66–10.42) | <.001 | 0.0c | 1.40 (0.78–2.53) | .260 | ||
Sex | |||||||||||
Male | 78.8 (55.7–100.0) | 1.00 | 45.7 (27.5–63.8) | 1.00 | 26.7 (14.2–39.2) | 1.00 | −0.11 (−0.54–0.32) | ||||
Female | 82.6 (59.4–95.8) | 1.02 (0.34–3.03) | .978 | 48.4 (36.8–60.1) | 1.37 (0.84–2.23) | .211 | 16.5 (5.6–27.5) | 0.99 (0.68–1.45) | .972 | ||
Ethnicity (n = 150) | |||||||||||
White | 88.3 (79.6–96.9) | 1.00 | 46.8 (34.2–59.4) | 1.00 | 21.9 (11.7–32.0) | 1.00 | 0.01 (−0.53–0.55) | .979 | |||
Nonwhite | 36.5 (0.0–89.5) | 2.43 (0.73–8.13) | .151 | 37.3 (15.6–59.1) | 1.49 (0.85–2.62) | .161 | 10.5 (0.0–27.4) | 1.24 (0.79–1.96) | .350 | ||
NF-1 | |||||||||||
negative | 78.5 (63.4–93.7) | 1.00 | 37.6 (24.9–50.3) | 1.00 | 13.0 (4.7–21.3) | 1.00 | −0.96 (−1.37 to –0.55) | <.001 | |||
Positive | 88.2 (73.6–100.0) | 0.52 (0.14–1.90) | .520 | 62.8 (45.0–80.7) | 0.43 (0.25–0.74) | .002 | 33.0 (15.3–50.8) | 0.45 (0.30–0.68) | <.001 | ||
Symptomatic | |||||||||||
No | 95.7 (87.6–100.0) | 1.00 | 73.9 (56.0–91.9) | 1.00 | 10.6 (0.0–37.4) | 1.00 | 1.00 (0.44–1.56) | .001 | |||
Yes | 80.2 (67.3–93.1) | 1.53 (0.20–11.96) | .686 | 44.0 (32.9–55.1) | 1.97 (0.85–4.56) | .114 | 20.1 (11.8–28.4) | 1.93 (1.06–3.52) | .032 | ||
Diencephalic syndrome | 1.40 (0.63–2.17) | <.001 | |||||||||
No | 80.8 (66.8–94.8) | 1.00 | 53.3 (42.5–64.1) | 1.00 | 23.5 (14.3–32.7) | 1.00 | |||||
Yes | 78.3 (50.8–100.0) | 1.99 (0.44–9.04) | .375 | 0.0b | 4.13 (2.19–7.79) | <.001 | 0.0c | 1.74 (0.95–3.18) | .073 | ||
Tumor stage | |||||||||||
MDC 1 | 89.1 (74.2–100.0) | 1.00 | 68.3 (33.1–100.0) | 1.00 | 54.4 (21.0–87.8) | 1.00 | 0.27 (0.05–0.49) | .019 | |||
MDC 2 | 82.8 (64.9–100.0) | 0.81 (0.16–4.23) | .806 | 48.7 (34.3–63.2) | 3.64 (1.29–10.30) | .015 | 13.5 (2.7–24.3) | 4.31 (2.05–9.08) | <.001 | ||
MDC 3/4 | 80.0 (55.2–100.0) | 0.71 (0.10–5.07) | .735 | 37.3 (13.8–60.9) | 4.00 (1.34–11.97) | .013 | 21.7 (6.4–37.0) | 3.45 (1.55–7.70) | .002 | ||
MDC other midline | 80.4 (62.7–98.0) | 1.82 (0.33–10.03) | .491 | 33.6 (12.7–54.6) | 4.93 (1.63–14.87) | .005 | 14.1 (0.0–29.2) | 3.94 (1.74–8.91) | .001 | ||
Hypothalamic involvement | 1.11 (0.71–1.52) | <.001 | |||||||||
No | 87.3 (76.4–98.3) | 1.00 | 60.3 (46.4–74.3) | 1.00 | 38.6 (25.9–51.3) | 1.00 | |||||
Yes | 73.1 (49.7–96.5) | 1.75 (0.59–5.20) | .318 | 27.9 (12.5–43.3) | 2.86 (1.75–4.67) | <.001 | 0.0c | 3.03 (2.04–4.48) | <.001 | ||
Leptomeningeal metastases | 1.46 (0.33–2.58) | .012 | |||||||||
No | 80.4 (67.4–93.3) | 1.00 | 48.7 (37.8–59.5) | 1.00 | 22.0 (13.3–30.7) | 1.00 | |||||
Yes | 100.0a | 0.05 (0.00-∞) | .630 | 0.0b | 2.09 (0.76–5.78) | .155 | 0.0c | 0.95 (0.35–2.59) | .922 | ||
Hydrocephalus | |||||||||||
No | 87.4 (86.8–87.1) | 1.00 | 54.5 (41.0–68.0) | 1.00 | 24.6 (12.6–36.5) | 1.00 | 0.47 (0.03–0.91) | .037 | |||
Yes | 72.1 (47.5–96.8) | 2.00 (0.67–5.99) | .213 | 35.0 (18.2–51.7) | 1.64 (1.01–2.66) | .045 | 14.9 (3.7–26.2) | 1.65 (1.13–2.42) | .010 | ||
Tumor grade (n = 63) | |||||||||||
Grade I | 87.0 (76.7–94.7) | 1.00 | 23.2 (6.93–39.5) | 1.00 | 10.6 (0.0–21.4) | 1.00 | 0.30 (−0.73–1.34) | .559 | |||
Grade II | 60.3 (10.6–100.0) | 2.71 (0.44–16.56) | .280 | 35.9 (6.2–65.5) | 1.02 (0.45–2.32) | .961 | 5.6 (0.0–20.0) | 1.86 (0.95–3.64) | .072 | ||
Primary treatment strategy | |||||||||||
Untreated | 91.9 (82.9–100.0) | 1.00 | 59.3 (38.4–80.3) | 1.00 | 33.3 (14.7–52.0) | 1.00 | |||||
Resection | 78.0 (55.6–100.0) | 0.70 (0.21–2.29) | .553 | 42.7 (27.9–57.4) | 1.39 (0.85–2.27) | .184 | 11.7 (1.0–22.5) | 1.76 (1.20–2.59) | .004 | 0.66 (0.21–1.11) | .004 |
Decompression | 68.7 (38.3–99.0) | 1.52 (0.50–4.65) | .466 | 33.3 (15.5–51.1) | 1.61 (0.98–2.63) | .060 | 9.5 (0.0–19.6) | 1.85 (1.25–2.74) | .002 | 0.58 (0.12–1.04) | .014 |
Radiotherapy | 71.8 (47.6–95.9) | 2.04 (0.68–6.11) | .201 | 40.9 (21.2–60.6) | 1.05 (0.62–1.77) | .865 | 4.1 (0.0–10.4) | 2.99 (2.00–4.46) | <.001 | 1.06 (0.60–1.51) | <.001 |
Chemotherapy | 84.7 (65.8–100.0) | 1.01 (0.28–3.67) | .989 | 39.9 (22.6–57.2) | 1.64 (0.97–2.76) | .063 | 9.1 (0.0–22.1) | 0.87 (0.56–1.33) | .515 | −0.13 (−0.62–0.36) | .603 |
Final treatment strategy | |||||||||||
Untreated | 100.0b | 1.00 | — | — | — | 34.3 (8.2–60.4) | 1.00 | ||||
Resection | 76.5 (57.3–95.7) | 1.06 (0.35–3.19) | .923 | — | — | — | 11.9 (1.8–22.0) | 1.68 (1.15–2.45) | .008 | 0.76 (0.33–1.19) | .001 |
Decompression | 68.1 (46.5–89.7) | 3.27 (1.00–10.66) | .049 | — | — | — | 10.1 (1.2–19.0) | 1.75 (1.20–2.55) | .004 | 0.64 (0.21–1.06) | .004 |
Radiotherapy | 73.5 (55.0–92.0) | 2.41 (0.74–7.86) | .145 | — | — | — | 3.0 (0.0–8.2) | 3.39 (2.28–5.04) | <.001 | 1.37 (0.99–1.75) | <.001 |
Chemotherapy | 75.3 (52.0–98.5) | 1.01 (0.33–3.10) | .986 | — | — | — | 13.8 (1.0–26.7) | 0.94 (0.64–1.39) | .751 | 0.21 (−0.24–0.65) | .355 |
Treatment era | |||||||||||
1980–1996 | 78.0 (63.7–92.4) | 1.00 | 51.8 (38.2–65.5) | 1.00 | 24.9 (12.4–37.4) | 1.00 | −0.38 (−0.63 to –0.13) | .003 | |||
1997–2004 | 88.7 (74.2–100.0) | 0.53 (0.14–2.00) | .529 | 39.8 (16.9–62.6) | 1.01 (0.57–1.78) | .968 | 20.5 (5.1–35.8) | 0.92 (0.59–1.45) | .728 | ||
2005–2010 | 97.1 (91.5–100.0) | 0.53 (0.06–4.80) | .534 | 53.8 (38.2–69.5) | 1.41 (0.77–2.61) | .269 | 19.2 (1.4–36.9) | 1.27 (0.80–2.04) | .316 | ||
No. progressions | — | 1.43 (1.08–1.90) | .012 | — | — | — | — | 1.16 (1.01–1.32) | .030 | 0.40 (0.22–0.58) | <.001 |
No. surgeries | — | 1.04 (0.96–1.14) | .327 | — | — | — | — | 1.04 (1.01–1.08) | .026 | 0.14 (0.08–0.19) | <.001 |
Independent Variable . | OS (95% CI) . | PFS (95% CI) . | EEFS (95% CI) . | EMS (95% CI) . | |||||||
---|---|---|---|---|---|---|---|---|---|---|---|
20-Year OS . | Crude HR . | P . | 20-Year PFS . | Crude HR . | P . | 20-Year EEFS . | Crude HR . | P . | Crude β . | P . | |
Age | |||||||||||
>5 y | 88.7 (76.1–100.0) | 1.00 | 53.4 (36.1–70.8) | 1.00 | 23.8 (13.2–34.5) | 1.00 | −0.02 (−0.07–0.04) | .600 | |||
1–5 y | 80.0 (60.6–99.3) | 1.05 (0.28–3.94) | .945 | 49.5 (34.4–64.5) | 1.17 (0.69–2.00) | .566 | 25.6 (11.1–40.0) | 0.64 (0.43–0.97) | .034 | ||
<1 y | 62.9 (32.6–93.1) | 5.29 (1.32–21.22) | .019 | 6.7 (0.0–23.2) | 5.26 (2.66–10.42) | <.001 | 0.0c | 1.40 (0.78–2.53) | .260 | ||
Sex | |||||||||||
Male | 78.8 (55.7–100.0) | 1.00 | 45.7 (27.5–63.8) | 1.00 | 26.7 (14.2–39.2) | 1.00 | −0.11 (−0.54–0.32) | ||||
Female | 82.6 (59.4–95.8) | 1.02 (0.34–3.03) | .978 | 48.4 (36.8–60.1) | 1.37 (0.84–2.23) | .211 | 16.5 (5.6–27.5) | 0.99 (0.68–1.45) | .972 | ||
Ethnicity (n = 150) | |||||||||||
White | 88.3 (79.6–96.9) | 1.00 | 46.8 (34.2–59.4) | 1.00 | 21.9 (11.7–32.0) | 1.00 | 0.01 (−0.53–0.55) | .979 | |||
Nonwhite | 36.5 (0.0–89.5) | 2.43 (0.73–8.13) | .151 | 37.3 (15.6–59.1) | 1.49 (0.85–2.62) | .161 | 10.5 (0.0–27.4) | 1.24 (0.79–1.96) | .350 | ||
NF-1 | |||||||||||
negative | 78.5 (63.4–93.7) | 1.00 | 37.6 (24.9–50.3) | 1.00 | 13.0 (4.7–21.3) | 1.00 | −0.96 (−1.37 to –0.55) | <.001 | |||
Positive | 88.2 (73.6–100.0) | 0.52 (0.14–1.90) | .520 | 62.8 (45.0–80.7) | 0.43 (0.25–0.74) | .002 | 33.0 (15.3–50.8) | 0.45 (0.30–0.68) | <.001 | ||
Symptomatic | |||||||||||
No | 95.7 (87.6–100.0) | 1.00 | 73.9 (56.0–91.9) | 1.00 | 10.6 (0.0–37.4) | 1.00 | 1.00 (0.44–1.56) | .001 | |||
Yes | 80.2 (67.3–93.1) | 1.53 (0.20–11.96) | .686 | 44.0 (32.9–55.1) | 1.97 (0.85–4.56) | .114 | 20.1 (11.8–28.4) | 1.93 (1.06–3.52) | .032 | ||
Diencephalic syndrome | 1.40 (0.63–2.17) | <.001 | |||||||||
No | 80.8 (66.8–94.8) | 1.00 | 53.3 (42.5–64.1) | 1.00 | 23.5 (14.3–32.7) | 1.00 | |||||
Yes | 78.3 (50.8–100.0) | 1.99 (0.44–9.04) | .375 | 0.0b | 4.13 (2.19–7.79) | <.001 | 0.0c | 1.74 (0.95–3.18) | .073 | ||
Tumor stage | |||||||||||
MDC 1 | 89.1 (74.2–100.0) | 1.00 | 68.3 (33.1–100.0) | 1.00 | 54.4 (21.0–87.8) | 1.00 | 0.27 (0.05–0.49) | .019 | |||
MDC 2 | 82.8 (64.9–100.0) | 0.81 (0.16–4.23) | .806 | 48.7 (34.3–63.2) | 3.64 (1.29–10.30) | .015 | 13.5 (2.7–24.3) | 4.31 (2.05–9.08) | <.001 | ||
MDC 3/4 | 80.0 (55.2–100.0) | 0.71 (0.10–5.07) | .735 | 37.3 (13.8–60.9) | 4.00 (1.34–11.97) | .013 | 21.7 (6.4–37.0) | 3.45 (1.55–7.70) | .002 | ||
MDC other midline | 80.4 (62.7–98.0) | 1.82 (0.33–10.03) | .491 | 33.6 (12.7–54.6) | 4.93 (1.63–14.87) | .005 | 14.1 (0.0–29.2) | 3.94 (1.74–8.91) | .001 | ||
Hypothalamic involvement | 1.11 (0.71–1.52) | <.001 | |||||||||
No | 87.3 (76.4–98.3) | 1.00 | 60.3 (46.4–74.3) | 1.00 | 38.6 (25.9–51.3) | 1.00 | |||||
Yes | 73.1 (49.7–96.5) | 1.75 (0.59–5.20) | .318 | 27.9 (12.5–43.3) | 2.86 (1.75–4.67) | <.001 | 0.0c | 3.03 (2.04–4.48) | <.001 | ||
Leptomeningeal metastases | 1.46 (0.33–2.58) | .012 | |||||||||
No | 80.4 (67.4–93.3) | 1.00 | 48.7 (37.8–59.5) | 1.00 | 22.0 (13.3–30.7) | 1.00 | |||||
Yes | 100.0a | 0.05 (0.00-∞) | .630 | 0.0b | 2.09 (0.76–5.78) | .155 | 0.0c | 0.95 (0.35–2.59) | .922 | ||
Hydrocephalus | |||||||||||
No | 87.4 (86.8–87.1) | 1.00 | 54.5 (41.0–68.0) | 1.00 | 24.6 (12.6–36.5) | 1.00 | 0.47 (0.03–0.91) | .037 | |||
Yes | 72.1 (47.5–96.8) | 2.00 (0.67–5.99) | .213 | 35.0 (18.2–51.7) | 1.64 (1.01–2.66) | .045 | 14.9 (3.7–26.2) | 1.65 (1.13–2.42) | .010 | ||
Tumor grade (n = 63) | |||||||||||
Grade I | 87.0 (76.7–94.7) | 1.00 | 23.2 (6.93–39.5) | 1.00 | 10.6 (0.0–21.4) | 1.00 | 0.30 (−0.73–1.34) | .559 | |||
Grade II | 60.3 (10.6–100.0) | 2.71 (0.44–16.56) | .280 | 35.9 (6.2–65.5) | 1.02 (0.45–2.32) | .961 | 5.6 (0.0–20.0) | 1.86 (0.95–3.64) | .072 | ||
Primary treatment strategy | |||||||||||
Untreated | 91.9 (82.9–100.0) | 1.00 | 59.3 (38.4–80.3) | 1.00 | 33.3 (14.7–52.0) | 1.00 | |||||
Resection | 78.0 (55.6–100.0) | 0.70 (0.21–2.29) | .553 | 42.7 (27.9–57.4) | 1.39 (0.85–2.27) | .184 | 11.7 (1.0–22.5) | 1.76 (1.20–2.59) | .004 | 0.66 (0.21–1.11) | .004 |
Decompression | 68.7 (38.3–99.0) | 1.52 (0.50–4.65) | .466 | 33.3 (15.5–51.1) | 1.61 (0.98–2.63) | .060 | 9.5 (0.0–19.6) | 1.85 (1.25–2.74) | .002 | 0.58 (0.12–1.04) | .014 |
Radiotherapy | 71.8 (47.6–95.9) | 2.04 (0.68–6.11) | .201 | 40.9 (21.2–60.6) | 1.05 (0.62–1.77) | .865 | 4.1 (0.0–10.4) | 2.99 (2.00–4.46) | <.001 | 1.06 (0.60–1.51) | <.001 |
Chemotherapy | 84.7 (65.8–100.0) | 1.01 (0.28–3.67) | .989 | 39.9 (22.6–57.2) | 1.64 (0.97–2.76) | .063 | 9.1 (0.0–22.1) | 0.87 (0.56–1.33) | .515 | −0.13 (−0.62–0.36) | .603 |
Final treatment strategy | |||||||||||
Untreated | 100.0b | 1.00 | — | — | — | 34.3 (8.2–60.4) | 1.00 | ||||
Resection | 76.5 (57.3–95.7) | 1.06 (0.35–3.19) | .923 | — | — | — | 11.9 (1.8–22.0) | 1.68 (1.15–2.45) | .008 | 0.76 (0.33–1.19) | .001 |
Decompression | 68.1 (46.5–89.7) | 3.27 (1.00–10.66) | .049 | — | — | — | 10.1 (1.2–19.0) | 1.75 (1.20–2.55) | .004 | 0.64 (0.21–1.06) | .004 |
Radiotherapy | 73.5 (55.0–92.0) | 2.41 (0.74–7.86) | .145 | — | — | — | 3.0 (0.0–8.2) | 3.39 (2.28–5.04) | <.001 | 1.37 (0.99–1.75) | <.001 |
Chemotherapy | 75.3 (52.0–98.5) | 1.01 (0.33–3.10) | .986 | — | — | — | 13.8 (1.0–26.7) | 0.94 (0.64–1.39) | .751 | 0.21 (−0.24–0.65) | .355 |
Treatment era | |||||||||||
1980–1996 | 78.0 (63.7–92.4) | 1.00 | 51.8 (38.2–65.5) | 1.00 | 24.9 (12.4–37.4) | 1.00 | −0.38 (−0.63 to –0.13) | .003 | |||
1997–2004 | 88.7 (74.2–100.0) | 0.53 (0.14–2.00) | .529 | 39.8 (16.9–62.6) | 1.01 (0.57–1.78) | .968 | 20.5 (5.1–35.8) | 0.92 (0.59–1.45) | .728 | ||
2005–2010 | 97.1 (91.5–100.0) | 0.53 (0.06–4.80) | .534 | 53.8 (38.2–69.5) | 1.41 (0.77–2.61) | .269 | 19.2 (1.4–36.9) | 1.27 (0.80–2.04) | .316 | ||
No. progressions | — | 1.43 (1.08–1.90) | .012 | — | — | — | — | 1.16 (1.01–1.32) | .030 | 0.40 (0.22–0.58) | <.001 |
No. surgeries | — | 1.04 (0.96–1.14) | .327 | — | — | — | — | 1.04 (1.01–1.08) | .026 | 0.14 (0.08–0.19) | <.001 |
Bold values indicate P < .05.
All patients with leptomeningeal metastases survived to last followup.
All patients with diencephalic syndrome and/ or leptomeningeal metastases progressed.
All patients aged <1 y at diagnosis, with diencephalic syndrome, hypothalamic involvement or leptomeningeal metastases experienced ≥1 endocrinopathy.
Twenty-Year OS, PFS, EEFS, and EMS at Last Followup With Crude HRs (95% CI) and Unadjusted Regression Coefficients β, Respectively
Independent Variable . | OS (95% CI) . | PFS (95% CI) . | EEFS (95% CI) . | EMS (95% CI) . | |||||||
---|---|---|---|---|---|---|---|---|---|---|---|
20-Year OS . | Crude HR . | P . | 20-Year PFS . | Crude HR . | P . | 20-Year EEFS . | Crude HR . | P . | Crude β . | P . | |
Age | |||||||||||
>5 y | 88.7 (76.1–100.0) | 1.00 | 53.4 (36.1–70.8) | 1.00 | 23.8 (13.2–34.5) | 1.00 | −0.02 (−0.07–0.04) | .600 | |||
1–5 y | 80.0 (60.6–99.3) | 1.05 (0.28–3.94) | .945 | 49.5 (34.4–64.5) | 1.17 (0.69–2.00) | .566 | 25.6 (11.1–40.0) | 0.64 (0.43–0.97) | .034 | ||
<1 y | 62.9 (32.6–93.1) | 5.29 (1.32–21.22) | .019 | 6.7 (0.0–23.2) | 5.26 (2.66–10.42) | <.001 | 0.0c | 1.40 (0.78–2.53) | .260 | ||
Sex | |||||||||||
Male | 78.8 (55.7–100.0) | 1.00 | 45.7 (27.5–63.8) | 1.00 | 26.7 (14.2–39.2) | 1.00 | −0.11 (−0.54–0.32) | ||||
Female | 82.6 (59.4–95.8) | 1.02 (0.34–3.03) | .978 | 48.4 (36.8–60.1) | 1.37 (0.84–2.23) | .211 | 16.5 (5.6–27.5) | 0.99 (0.68–1.45) | .972 | ||
Ethnicity (n = 150) | |||||||||||
White | 88.3 (79.6–96.9) | 1.00 | 46.8 (34.2–59.4) | 1.00 | 21.9 (11.7–32.0) | 1.00 | 0.01 (−0.53–0.55) | .979 | |||
Nonwhite | 36.5 (0.0–89.5) | 2.43 (0.73–8.13) | .151 | 37.3 (15.6–59.1) | 1.49 (0.85–2.62) | .161 | 10.5 (0.0–27.4) | 1.24 (0.79–1.96) | .350 | ||
NF-1 | |||||||||||
negative | 78.5 (63.4–93.7) | 1.00 | 37.6 (24.9–50.3) | 1.00 | 13.0 (4.7–21.3) | 1.00 | −0.96 (−1.37 to –0.55) | <.001 | |||
Positive | 88.2 (73.6–100.0) | 0.52 (0.14–1.90) | .520 | 62.8 (45.0–80.7) | 0.43 (0.25–0.74) | .002 | 33.0 (15.3–50.8) | 0.45 (0.30–0.68) | <.001 | ||
Symptomatic | |||||||||||
No | 95.7 (87.6–100.0) | 1.00 | 73.9 (56.0–91.9) | 1.00 | 10.6 (0.0–37.4) | 1.00 | 1.00 (0.44–1.56) | .001 | |||
Yes | 80.2 (67.3–93.1) | 1.53 (0.20–11.96) | .686 | 44.0 (32.9–55.1) | 1.97 (0.85–4.56) | .114 | 20.1 (11.8–28.4) | 1.93 (1.06–3.52) | .032 | ||
Diencephalic syndrome | 1.40 (0.63–2.17) | <.001 | |||||||||
No | 80.8 (66.8–94.8) | 1.00 | 53.3 (42.5–64.1) | 1.00 | 23.5 (14.3–32.7) | 1.00 | |||||
Yes | 78.3 (50.8–100.0) | 1.99 (0.44–9.04) | .375 | 0.0b | 4.13 (2.19–7.79) | <.001 | 0.0c | 1.74 (0.95–3.18) | .073 | ||
Tumor stage | |||||||||||
MDC 1 | 89.1 (74.2–100.0) | 1.00 | 68.3 (33.1–100.0) | 1.00 | 54.4 (21.0–87.8) | 1.00 | 0.27 (0.05–0.49) | .019 | |||
MDC 2 | 82.8 (64.9–100.0) | 0.81 (0.16–4.23) | .806 | 48.7 (34.3–63.2) | 3.64 (1.29–10.30) | .015 | 13.5 (2.7–24.3) | 4.31 (2.05–9.08) | <.001 | ||
MDC 3/4 | 80.0 (55.2–100.0) | 0.71 (0.10–5.07) | .735 | 37.3 (13.8–60.9) | 4.00 (1.34–11.97) | .013 | 21.7 (6.4–37.0) | 3.45 (1.55–7.70) | .002 | ||
MDC other midline | 80.4 (62.7–98.0) | 1.82 (0.33–10.03) | .491 | 33.6 (12.7–54.6) | 4.93 (1.63–14.87) | .005 | 14.1 (0.0–29.2) | 3.94 (1.74–8.91) | .001 | ||
Hypothalamic involvement | 1.11 (0.71–1.52) | <.001 | |||||||||
No | 87.3 (76.4–98.3) | 1.00 | 60.3 (46.4–74.3) | 1.00 | 38.6 (25.9–51.3) | 1.00 | |||||
Yes | 73.1 (49.7–96.5) | 1.75 (0.59–5.20) | .318 | 27.9 (12.5–43.3) | 2.86 (1.75–4.67) | <.001 | 0.0c | 3.03 (2.04–4.48) | <.001 | ||
Leptomeningeal metastases | 1.46 (0.33–2.58) | .012 | |||||||||
No | 80.4 (67.4–93.3) | 1.00 | 48.7 (37.8–59.5) | 1.00 | 22.0 (13.3–30.7) | 1.00 | |||||
Yes | 100.0a | 0.05 (0.00-∞) | .630 | 0.0b | 2.09 (0.76–5.78) | .155 | 0.0c | 0.95 (0.35–2.59) | .922 | ||
Hydrocephalus | |||||||||||
No | 87.4 (86.8–87.1) | 1.00 | 54.5 (41.0–68.0) | 1.00 | 24.6 (12.6–36.5) | 1.00 | 0.47 (0.03–0.91) | .037 | |||
Yes | 72.1 (47.5–96.8) | 2.00 (0.67–5.99) | .213 | 35.0 (18.2–51.7) | 1.64 (1.01–2.66) | .045 | 14.9 (3.7–26.2) | 1.65 (1.13–2.42) | .010 | ||
Tumor grade (n = 63) | |||||||||||
Grade I | 87.0 (76.7–94.7) | 1.00 | 23.2 (6.93–39.5) | 1.00 | 10.6 (0.0–21.4) | 1.00 | 0.30 (−0.73–1.34) | .559 | |||
Grade II | 60.3 (10.6–100.0) | 2.71 (0.44–16.56) | .280 | 35.9 (6.2–65.5) | 1.02 (0.45–2.32) | .961 | 5.6 (0.0–20.0) | 1.86 (0.95–3.64) | .072 | ||
Primary treatment strategy | |||||||||||
Untreated | 91.9 (82.9–100.0) | 1.00 | 59.3 (38.4–80.3) | 1.00 | 33.3 (14.7–52.0) | 1.00 | |||||
Resection | 78.0 (55.6–100.0) | 0.70 (0.21–2.29) | .553 | 42.7 (27.9–57.4) | 1.39 (0.85–2.27) | .184 | 11.7 (1.0–22.5) | 1.76 (1.20–2.59) | .004 | 0.66 (0.21–1.11) | .004 |
Decompression | 68.7 (38.3–99.0) | 1.52 (0.50–4.65) | .466 | 33.3 (15.5–51.1) | 1.61 (0.98–2.63) | .060 | 9.5 (0.0–19.6) | 1.85 (1.25–2.74) | .002 | 0.58 (0.12–1.04) | .014 |
Radiotherapy | 71.8 (47.6–95.9) | 2.04 (0.68–6.11) | .201 | 40.9 (21.2–60.6) | 1.05 (0.62–1.77) | .865 | 4.1 (0.0–10.4) | 2.99 (2.00–4.46) | <.001 | 1.06 (0.60–1.51) | <.001 |
Chemotherapy | 84.7 (65.8–100.0) | 1.01 (0.28–3.67) | .989 | 39.9 (22.6–57.2) | 1.64 (0.97–2.76) | .063 | 9.1 (0.0–22.1) | 0.87 (0.56–1.33) | .515 | −0.13 (−0.62–0.36) | .603 |
Final treatment strategy | |||||||||||
Untreated | 100.0b | 1.00 | — | — | — | 34.3 (8.2–60.4) | 1.00 | ||||
Resection | 76.5 (57.3–95.7) | 1.06 (0.35–3.19) | .923 | — | — | — | 11.9 (1.8–22.0) | 1.68 (1.15–2.45) | .008 | 0.76 (0.33–1.19) | .001 |
Decompression | 68.1 (46.5–89.7) | 3.27 (1.00–10.66) | .049 | — | — | — | 10.1 (1.2–19.0) | 1.75 (1.20–2.55) | .004 | 0.64 (0.21–1.06) | .004 |
Radiotherapy | 73.5 (55.0–92.0) | 2.41 (0.74–7.86) | .145 | — | — | — | 3.0 (0.0–8.2) | 3.39 (2.28–5.04) | <.001 | 1.37 (0.99–1.75) | <.001 |
Chemotherapy | 75.3 (52.0–98.5) | 1.01 (0.33–3.10) | .986 | — | — | — | 13.8 (1.0–26.7) | 0.94 (0.64–1.39) | .751 | 0.21 (−0.24–0.65) | .355 |
Treatment era | |||||||||||
1980–1996 | 78.0 (63.7–92.4) | 1.00 | 51.8 (38.2–65.5) | 1.00 | 24.9 (12.4–37.4) | 1.00 | −0.38 (−0.63 to –0.13) | .003 | |||
1997–2004 | 88.7 (74.2–100.0) | 0.53 (0.14–2.00) | .529 | 39.8 (16.9–62.6) | 1.01 (0.57–1.78) | .968 | 20.5 (5.1–35.8) | 0.92 (0.59–1.45) | .728 | ||
2005–2010 | 97.1 (91.5–100.0) | 0.53 (0.06–4.80) | .534 | 53.8 (38.2–69.5) | 1.41 (0.77–2.61) | .269 | 19.2 (1.4–36.9) | 1.27 (0.80–2.04) | .316 | ||
No. progressions | — | 1.43 (1.08–1.90) | .012 | — | — | — | — | 1.16 (1.01–1.32) | .030 | 0.40 (0.22–0.58) | <.001 |
No. surgeries | — | 1.04 (0.96–1.14) | .327 | — | — | — | — | 1.04 (1.01–1.08) | .026 | 0.14 (0.08–0.19) | <.001 |
Independent Variable . | OS (95% CI) . | PFS (95% CI) . | EEFS (95% CI) . | EMS (95% CI) . | |||||||
---|---|---|---|---|---|---|---|---|---|---|---|
20-Year OS . | Crude HR . | P . | 20-Year PFS . | Crude HR . | P . | 20-Year EEFS . | Crude HR . | P . | Crude β . | P . | |
Age | |||||||||||
>5 y | 88.7 (76.1–100.0) | 1.00 | 53.4 (36.1–70.8) | 1.00 | 23.8 (13.2–34.5) | 1.00 | −0.02 (−0.07–0.04) | .600 | |||
1–5 y | 80.0 (60.6–99.3) | 1.05 (0.28–3.94) | .945 | 49.5 (34.4–64.5) | 1.17 (0.69–2.00) | .566 | 25.6 (11.1–40.0) | 0.64 (0.43–0.97) | .034 | ||
<1 y | 62.9 (32.6–93.1) | 5.29 (1.32–21.22) | .019 | 6.7 (0.0–23.2) | 5.26 (2.66–10.42) | <.001 | 0.0c | 1.40 (0.78–2.53) | .260 | ||
Sex | |||||||||||
Male | 78.8 (55.7–100.0) | 1.00 | 45.7 (27.5–63.8) | 1.00 | 26.7 (14.2–39.2) | 1.00 | −0.11 (−0.54–0.32) | ||||
Female | 82.6 (59.4–95.8) | 1.02 (0.34–3.03) | .978 | 48.4 (36.8–60.1) | 1.37 (0.84–2.23) | .211 | 16.5 (5.6–27.5) | 0.99 (0.68–1.45) | .972 | ||
Ethnicity (n = 150) | |||||||||||
White | 88.3 (79.6–96.9) | 1.00 | 46.8 (34.2–59.4) | 1.00 | 21.9 (11.7–32.0) | 1.00 | 0.01 (−0.53–0.55) | .979 | |||
Nonwhite | 36.5 (0.0–89.5) | 2.43 (0.73–8.13) | .151 | 37.3 (15.6–59.1) | 1.49 (0.85–2.62) | .161 | 10.5 (0.0–27.4) | 1.24 (0.79–1.96) | .350 | ||
NF-1 | |||||||||||
negative | 78.5 (63.4–93.7) | 1.00 | 37.6 (24.9–50.3) | 1.00 | 13.0 (4.7–21.3) | 1.00 | −0.96 (−1.37 to –0.55) | <.001 | |||
Positive | 88.2 (73.6–100.0) | 0.52 (0.14–1.90) | .520 | 62.8 (45.0–80.7) | 0.43 (0.25–0.74) | .002 | 33.0 (15.3–50.8) | 0.45 (0.30–0.68) | <.001 | ||
Symptomatic | |||||||||||
No | 95.7 (87.6–100.0) | 1.00 | 73.9 (56.0–91.9) | 1.00 | 10.6 (0.0–37.4) | 1.00 | 1.00 (0.44–1.56) | .001 | |||
Yes | 80.2 (67.3–93.1) | 1.53 (0.20–11.96) | .686 | 44.0 (32.9–55.1) | 1.97 (0.85–4.56) | .114 | 20.1 (11.8–28.4) | 1.93 (1.06–3.52) | .032 | ||
Diencephalic syndrome | 1.40 (0.63–2.17) | <.001 | |||||||||
No | 80.8 (66.8–94.8) | 1.00 | 53.3 (42.5–64.1) | 1.00 | 23.5 (14.3–32.7) | 1.00 | |||||
Yes | 78.3 (50.8–100.0) | 1.99 (0.44–9.04) | .375 | 0.0b | 4.13 (2.19–7.79) | <.001 | 0.0c | 1.74 (0.95–3.18) | .073 | ||
Tumor stage | |||||||||||
MDC 1 | 89.1 (74.2–100.0) | 1.00 | 68.3 (33.1–100.0) | 1.00 | 54.4 (21.0–87.8) | 1.00 | 0.27 (0.05–0.49) | .019 | |||
MDC 2 | 82.8 (64.9–100.0) | 0.81 (0.16–4.23) | .806 | 48.7 (34.3–63.2) | 3.64 (1.29–10.30) | .015 | 13.5 (2.7–24.3) | 4.31 (2.05–9.08) | <.001 | ||
MDC 3/4 | 80.0 (55.2–100.0) | 0.71 (0.10–5.07) | .735 | 37.3 (13.8–60.9) | 4.00 (1.34–11.97) | .013 | 21.7 (6.4–37.0) | 3.45 (1.55–7.70) | .002 | ||
MDC other midline | 80.4 (62.7–98.0) | 1.82 (0.33–10.03) | .491 | 33.6 (12.7–54.6) | 4.93 (1.63–14.87) | .005 | 14.1 (0.0–29.2) | 3.94 (1.74–8.91) | .001 | ||
Hypothalamic involvement | 1.11 (0.71–1.52) | <.001 | |||||||||
No | 87.3 (76.4–98.3) | 1.00 | 60.3 (46.4–74.3) | 1.00 | 38.6 (25.9–51.3) | 1.00 | |||||
Yes | 73.1 (49.7–96.5) | 1.75 (0.59–5.20) | .318 | 27.9 (12.5–43.3) | 2.86 (1.75–4.67) | <.001 | 0.0c | 3.03 (2.04–4.48) | <.001 | ||
Leptomeningeal metastases | 1.46 (0.33–2.58) | .012 | |||||||||
No | 80.4 (67.4–93.3) | 1.00 | 48.7 (37.8–59.5) | 1.00 | 22.0 (13.3–30.7) | 1.00 | |||||
Yes | 100.0a | 0.05 (0.00-∞) | .630 | 0.0b | 2.09 (0.76–5.78) | .155 | 0.0c | 0.95 (0.35–2.59) | .922 | ||
Hydrocephalus | |||||||||||
No | 87.4 (86.8–87.1) | 1.00 | 54.5 (41.0–68.0) | 1.00 | 24.6 (12.6–36.5) | 1.00 | 0.47 (0.03–0.91) | .037 | |||
Yes | 72.1 (47.5–96.8) | 2.00 (0.67–5.99) | .213 | 35.0 (18.2–51.7) | 1.64 (1.01–2.66) | .045 | 14.9 (3.7–26.2) | 1.65 (1.13–2.42) | .010 | ||
Tumor grade (n = 63) | |||||||||||
Grade I | 87.0 (76.7–94.7) | 1.00 | 23.2 (6.93–39.5) | 1.00 | 10.6 (0.0–21.4) | 1.00 | 0.30 (−0.73–1.34) | .559 | |||
Grade II | 60.3 (10.6–100.0) | 2.71 (0.44–16.56) | .280 | 35.9 (6.2–65.5) | 1.02 (0.45–2.32) | .961 | 5.6 (0.0–20.0) | 1.86 (0.95–3.64) | .072 | ||
Primary treatment strategy | |||||||||||
Untreated | 91.9 (82.9–100.0) | 1.00 | 59.3 (38.4–80.3) | 1.00 | 33.3 (14.7–52.0) | 1.00 | |||||
Resection | 78.0 (55.6–100.0) | 0.70 (0.21–2.29) | .553 | 42.7 (27.9–57.4) | 1.39 (0.85–2.27) | .184 | 11.7 (1.0–22.5) | 1.76 (1.20–2.59) | .004 | 0.66 (0.21–1.11) | .004 |
Decompression | 68.7 (38.3–99.0) | 1.52 (0.50–4.65) | .466 | 33.3 (15.5–51.1) | 1.61 (0.98–2.63) | .060 | 9.5 (0.0–19.6) | 1.85 (1.25–2.74) | .002 | 0.58 (0.12–1.04) | .014 |
Radiotherapy | 71.8 (47.6–95.9) | 2.04 (0.68–6.11) | .201 | 40.9 (21.2–60.6) | 1.05 (0.62–1.77) | .865 | 4.1 (0.0–10.4) | 2.99 (2.00–4.46) | <.001 | 1.06 (0.60–1.51) | <.001 |
Chemotherapy | 84.7 (65.8–100.0) | 1.01 (0.28–3.67) | .989 | 39.9 (22.6–57.2) | 1.64 (0.97–2.76) | .063 | 9.1 (0.0–22.1) | 0.87 (0.56–1.33) | .515 | −0.13 (−0.62–0.36) | .603 |
Final treatment strategy | |||||||||||
Untreated | 100.0b | 1.00 | — | — | — | 34.3 (8.2–60.4) | 1.00 | ||||
Resection | 76.5 (57.3–95.7) | 1.06 (0.35–3.19) | .923 | — | — | — | 11.9 (1.8–22.0) | 1.68 (1.15–2.45) | .008 | 0.76 (0.33–1.19) | .001 |
Decompression | 68.1 (46.5–89.7) | 3.27 (1.00–10.66) | .049 | — | — | — | 10.1 (1.2–19.0) | 1.75 (1.20–2.55) | .004 | 0.64 (0.21–1.06) | .004 |
Radiotherapy | 73.5 (55.0–92.0) | 2.41 (0.74–7.86) | .145 | — | — | — | 3.0 (0.0–8.2) | 3.39 (2.28–5.04) | <.001 | 1.37 (0.99–1.75) | <.001 |
Chemotherapy | 75.3 (52.0–98.5) | 1.01 (0.33–3.10) | .986 | — | — | — | 13.8 (1.0–26.7) | 0.94 (0.64–1.39) | .751 | 0.21 (−0.24–0.65) | .355 |
Treatment era | |||||||||||
1980–1996 | 78.0 (63.7–92.4) | 1.00 | 51.8 (38.2–65.5) | 1.00 | 24.9 (12.4–37.4) | 1.00 | −0.38 (−0.63 to –0.13) | .003 | |||
1997–2004 | 88.7 (74.2–100.0) | 0.53 (0.14–2.00) | .529 | 39.8 (16.9–62.6) | 1.01 (0.57–1.78) | .968 | 20.5 (5.1–35.8) | 0.92 (0.59–1.45) | .728 | ||
2005–2010 | 97.1 (91.5–100.0) | 0.53 (0.06–4.80) | .534 | 53.8 (38.2–69.5) | 1.41 (0.77–2.61) | .269 | 19.2 (1.4–36.9) | 1.27 (0.80–2.04) | .316 | ||
No. progressions | — | 1.43 (1.08–1.90) | .012 | — | — | — | — | 1.16 (1.01–1.32) | .030 | 0.40 (0.22–0.58) | <.001 |
No. surgeries | — | 1.04 (0.96–1.14) | .327 | — | — | — | — | 1.04 (1.01–1.08) | .026 | 0.14 (0.08–0.19) | <.001 |
Bold values indicate P < .05.
All patients with leptomeningeal metastases survived to last followup.
All patients with diencephalic syndrome and/ or leptomeningeal metastases progressed.
All patients aged <1 y at diagnosis, with diencephalic syndrome, hypothalamic involvement or leptomeningeal metastases experienced ≥1 endocrinopathy.
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