Table 2.

Twenty-Year OS, PFS, EEFS, and EMS at Last Followup With Crude HRs (95% CI) and Unadjusted Regression Coefficients β, Respectively

Independent VariableOS (95% CI)PFS (95% CI)EEFS (95% CI)EMS (95% CI)
20-Year OSCrude HRP20-Year PFSCrude HRP20-Year EEFSCrude HRPCrude βP
Age
    >5 y88.7 (76.1–100.0)1.0053.4 (36.1–70.8)1.0023.8 (13.2–34.5)1.00−0.02 (−0.07–0.04).600
    1–5 y80.0 (60.6–99.3)1.05 (0.28–3.94).94549.5 (34.4–64.5)1.17 (0.69–2.00).56625.6 (11.1–40.0)0.64 (0.43–0.97).034
    <1 y62.9 (32.6–93.1)5.29 (1.32–21.22).0196.7 (0.0–23.2)5.26 (2.66–10.42)<.0010.0c1.40 (0.78–2.53).260
Sex
    Male78.8 (55.7–100.0)1.0045.7 (27.5–63.8)1.0026.7 (14.2–39.2)1.00−0.11 (−0.54–0.32)
    Female82.6 (59.4–95.8)1.02 (0.34–3.03).97848.4 (36.8–60.1)1.37 (0.84–2.23).21116.5 (5.6–27.5)0.99 (0.68–1.45).972
Ethnicity (n = 150)
    White88.3 (79.6–96.9)1.0046.8 (34.2–59.4)1.0021.9 (11.7–32.0)1.000.01 (−0.53–0.55).979
    Nonwhite36.5 (0.0–89.5)2.43 (0.73–8.13).15137.3 (15.6–59.1)1.49 (0.85–2.62).16110.5 (0.0–27.4)1.24 (0.79–1.96).350
NF-1
    negative78.5 (63.4–93.7)1.0037.6 (24.9–50.3)1.0013.0 (4.7–21.3)1.00−0.96 (−1.37 to –0.55)<.001
    Positive88.2 (73.6–100.0)0.52 (0.14–1.90).52062.8 (45.0–80.7)0.43 (0.25–0.74).00233.0 (15.3–50.8)0.45 (0.30–0.68)<.001
Symptomatic
    No95.7 (87.6–100.0)1.0073.9 (56.0–91.9)1.0010.6 (0.0–37.4)1.001.00 (0.44–1.56).001
    Yes80.2 (67.3–93.1)1.53 (0.20–11.96).68644.0 (32.9–55.1)1.97 (0.85–4.56).11420.1 (11.8–28.4)1.93 (1.06–3.52).032
Diencephalic syndrome1.40 (0.63–2.17)<.001
    No80.8 (66.8–94.8)1.0053.3 (42.5–64.1)1.0023.5 (14.3–32.7)1.00
    Yes78.3 (50.8–100.0)1.99 (0.44–9.04).3750.0b4.13 (2.19–7.79)<.0010.0c1.74 (0.95–3.18).073
Tumor stage
    MDC 189.1 (74.2–100.0)1.0068.3 (33.1–100.0)1.0054.4 (21.0–87.8)1.000.27 (0.05–0.49).019
    MDC 282.8 (64.9–100.0)0.81 (0.16–4.23).80648.7 (34.3–63.2)3.64 (1.29–10.30).01513.5 (2.7–24.3)4.31 (2.05–9.08)<.001
    MDC 3/480.0 (55.2–100.0)0.71 (0.10–5.07).73537.3 (13.8–60.9)4.00 (1.34–11.97).01321.7 (6.4–37.0)3.45 (1.55–7.70).002
    MDC other midline80.4 (62.7–98.0)1.82 (0.33–10.03).49133.6 (12.7–54.6)4.93 (1.63–14.87).00514.1 (0.0–29.2)3.94 (1.74–8.91).001
Hypothalamic involvement1.11 (0.71–1.52)<.001
    No87.3 (76.4–98.3)1.0060.3 (46.4–74.3)1.0038.6 (25.9–51.3)1.00
    Yes73.1 (49.7–96.5)1.75 (0.59–5.20).31827.9 (12.5–43.3)2.86 (1.75–4.67)<.0010.0c3.03 (2.04–4.48)<.001
Leptomeningeal metastases1.46 (0.33–2.58).012
    No80.4 (67.4–93.3)1.0048.7 (37.8–59.5)1.0022.0 (13.3–30.7)1.00
    Yes100.0a0.05 (0.00-∞).6300.0b2.09 (0.76–5.78).1550.0c0.95 (0.35–2.59).922
Hydrocephalus
    No87.4 (86.8–87.1)1.0054.5 (41.0–68.0)1.0024.6 (12.6–36.5)1.000.47 (0.03–0.91).037
    Yes72.1 (47.5–96.8)2.00 (0.67–5.99).21335.0 (18.2–51.7)1.64 (1.01–2.66).04514.9 (3.7–26.2)1.65 (1.13–2.42).010
Tumor grade (n = 63)
    Grade I87.0 (76.7–94.7)1.0023.2 (6.93–39.5)1.0010.6 (0.0–21.4)1.000.30 (−0.73–1.34).559
    Grade II60.3 (10.6–100.0)2.71 (0.44–16.56).28035.9 (6.2–65.5)1.02 (0.45–2.32).9615.6 (0.0–20.0)1.86 (0.95–3.64).072
Primary treatment strategy
    Untreated91.9 (82.9–100.0)1.0059.3 (38.4–80.3)1.0033.3 (14.7–52.0)1.00
    Resection78.0 (55.6–100.0)0.70 (0.21–2.29).55342.7 (27.9–57.4)1.39 (0.85–2.27).18411.7 (1.0–22.5)1.76 (1.20–2.59).0040.66 (0.21–1.11).004
    Decompression68.7 (38.3–99.0)1.52 (0.50–4.65).46633.3 (15.5–51.1)1.61 (0.98–2.63).0609.5 (0.0–19.6)1.85 (1.25–2.74).0020.58 (0.12–1.04).014
    Radiotherapy71.8 (47.6–95.9)2.04 (0.68–6.11).20140.9 (21.2–60.6)1.05 (0.62–1.77).8654.1 (0.0–10.4)2.99 (2.00–4.46)<.0011.06 (0.60–1.51)<.001
    Chemotherapy84.7 (65.8–100.0)1.01 (0.28–3.67).98939.9 (22.6–57.2)1.64 (0.97–2.76).0639.1 (0.0–22.1)0.87 (0.56–1.33).515−0.13 (−0.62–0.36).603
Final treatment strategy
    Untreated100.0b1.0034.3 (8.2–60.4)1.00
    Resection76.5 (57.3–95.7)1.06 (0.35–3.19).92311.9 (1.8–22.0)1.68 (1.15–2.45).0080.76 (0.33–1.19).001
    Decompression68.1 (46.5–89.7)3.27 (1.00–10.66).04910.1 (1.2–19.0)1.75 (1.20–2.55).0040.64 (0.21–1.06).004
    Radiotherapy73.5 (55.0–92.0)2.41 (0.74–7.86).1453.0 (0.0–8.2)3.39 (2.28–5.04)<.0011.37 (0.99–1.75)<.001
    Chemotherapy75.3 (52.0–98.5)1.01 (0.33–3.10).98613.8 (1.0–26.7)0.94 (0.64–1.39).7510.21 (−0.24–0.65).355
Treatment era
    1980–199678.0 (63.7–92.4)1.0051.8 (38.2–65.5)1.0024.9 (12.4–37.4)1.00−0.38 (−0.63 to –0.13).003
    1997–200488.7 (74.2–100.0)0.53 (0.14–2.00).52939.8 (16.9–62.6)1.01 (0.57–1.78).96820.5 (5.1–35.8)0.92 (0.59–1.45).728
    2005–201097.1 (91.5–100.0)0.53 (0.06–4.80).53453.8 (38.2–69.5)1.41 (0.77–2.61).26919.2 (1.4–36.9)1.27 (0.80–2.04).316
No. progressions1.43 (1.08–1.90).0121.16 (1.01–1.32).0300.40 (0.22–0.58)<.001
No. surgeries1.04 (0.96–1.14).3271.04 (1.01–1.08).0260.14 (0.08–0.19)<.001
Independent VariableOS (95% CI)PFS (95% CI)EEFS (95% CI)EMS (95% CI)
20-Year OSCrude HRP20-Year PFSCrude HRP20-Year EEFSCrude HRPCrude βP
Age
    >5 y88.7 (76.1–100.0)1.0053.4 (36.1–70.8)1.0023.8 (13.2–34.5)1.00−0.02 (−0.07–0.04).600
    1–5 y80.0 (60.6–99.3)1.05 (0.28–3.94).94549.5 (34.4–64.5)1.17 (0.69–2.00).56625.6 (11.1–40.0)0.64 (0.43–0.97).034
    <1 y62.9 (32.6–93.1)5.29 (1.32–21.22).0196.7 (0.0–23.2)5.26 (2.66–10.42)<.0010.0c1.40 (0.78–2.53).260
Sex
    Male78.8 (55.7–100.0)1.0045.7 (27.5–63.8)1.0026.7 (14.2–39.2)1.00−0.11 (−0.54–0.32)
    Female82.6 (59.4–95.8)1.02 (0.34–3.03).97848.4 (36.8–60.1)1.37 (0.84–2.23).21116.5 (5.6–27.5)0.99 (0.68–1.45).972
Ethnicity (n = 150)
    White88.3 (79.6–96.9)1.0046.8 (34.2–59.4)1.0021.9 (11.7–32.0)1.000.01 (−0.53–0.55).979
    Nonwhite36.5 (0.0–89.5)2.43 (0.73–8.13).15137.3 (15.6–59.1)1.49 (0.85–2.62).16110.5 (0.0–27.4)1.24 (0.79–1.96).350
NF-1
    negative78.5 (63.4–93.7)1.0037.6 (24.9–50.3)1.0013.0 (4.7–21.3)1.00−0.96 (−1.37 to –0.55)<.001
    Positive88.2 (73.6–100.0)0.52 (0.14–1.90).52062.8 (45.0–80.7)0.43 (0.25–0.74).00233.0 (15.3–50.8)0.45 (0.30–0.68)<.001
Symptomatic
    No95.7 (87.6–100.0)1.0073.9 (56.0–91.9)1.0010.6 (0.0–37.4)1.001.00 (0.44–1.56).001
    Yes80.2 (67.3–93.1)1.53 (0.20–11.96).68644.0 (32.9–55.1)1.97 (0.85–4.56).11420.1 (11.8–28.4)1.93 (1.06–3.52).032
Diencephalic syndrome1.40 (0.63–2.17)<.001
    No80.8 (66.8–94.8)1.0053.3 (42.5–64.1)1.0023.5 (14.3–32.7)1.00
    Yes78.3 (50.8–100.0)1.99 (0.44–9.04).3750.0b4.13 (2.19–7.79)<.0010.0c1.74 (0.95–3.18).073
Tumor stage
    MDC 189.1 (74.2–100.0)1.0068.3 (33.1–100.0)1.0054.4 (21.0–87.8)1.000.27 (0.05–0.49).019
    MDC 282.8 (64.9–100.0)0.81 (0.16–4.23).80648.7 (34.3–63.2)3.64 (1.29–10.30).01513.5 (2.7–24.3)4.31 (2.05–9.08)<.001
    MDC 3/480.0 (55.2–100.0)0.71 (0.10–5.07).73537.3 (13.8–60.9)4.00 (1.34–11.97).01321.7 (6.4–37.0)3.45 (1.55–7.70).002
    MDC other midline80.4 (62.7–98.0)1.82 (0.33–10.03).49133.6 (12.7–54.6)4.93 (1.63–14.87).00514.1 (0.0–29.2)3.94 (1.74–8.91).001
Hypothalamic involvement1.11 (0.71–1.52)<.001
    No87.3 (76.4–98.3)1.0060.3 (46.4–74.3)1.0038.6 (25.9–51.3)1.00
    Yes73.1 (49.7–96.5)1.75 (0.59–5.20).31827.9 (12.5–43.3)2.86 (1.75–4.67)<.0010.0c3.03 (2.04–4.48)<.001
Leptomeningeal metastases1.46 (0.33–2.58).012
    No80.4 (67.4–93.3)1.0048.7 (37.8–59.5)1.0022.0 (13.3–30.7)1.00
    Yes100.0a0.05 (0.00-∞).6300.0b2.09 (0.76–5.78).1550.0c0.95 (0.35–2.59).922
Hydrocephalus
    No87.4 (86.8–87.1)1.0054.5 (41.0–68.0)1.0024.6 (12.6–36.5)1.000.47 (0.03–0.91).037
    Yes72.1 (47.5–96.8)2.00 (0.67–5.99).21335.0 (18.2–51.7)1.64 (1.01–2.66).04514.9 (3.7–26.2)1.65 (1.13–2.42).010
Tumor grade (n = 63)
    Grade I87.0 (76.7–94.7)1.0023.2 (6.93–39.5)1.0010.6 (0.0–21.4)1.000.30 (−0.73–1.34).559
    Grade II60.3 (10.6–100.0)2.71 (0.44–16.56).28035.9 (6.2–65.5)1.02 (0.45–2.32).9615.6 (0.0–20.0)1.86 (0.95–3.64).072
Primary treatment strategy
    Untreated91.9 (82.9–100.0)1.0059.3 (38.4–80.3)1.0033.3 (14.7–52.0)1.00
    Resection78.0 (55.6–100.0)0.70 (0.21–2.29).55342.7 (27.9–57.4)1.39 (0.85–2.27).18411.7 (1.0–22.5)1.76 (1.20–2.59).0040.66 (0.21–1.11).004
    Decompression68.7 (38.3–99.0)1.52 (0.50–4.65).46633.3 (15.5–51.1)1.61 (0.98–2.63).0609.5 (0.0–19.6)1.85 (1.25–2.74).0020.58 (0.12–1.04).014
    Radiotherapy71.8 (47.6–95.9)2.04 (0.68–6.11).20140.9 (21.2–60.6)1.05 (0.62–1.77).8654.1 (0.0–10.4)2.99 (2.00–4.46)<.0011.06 (0.60–1.51)<.001
    Chemotherapy84.7 (65.8–100.0)1.01 (0.28–3.67).98939.9 (22.6–57.2)1.64 (0.97–2.76).0639.1 (0.0–22.1)0.87 (0.56–1.33).515−0.13 (−0.62–0.36).603
Final treatment strategy
    Untreated100.0b1.0034.3 (8.2–60.4)1.00
    Resection76.5 (57.3–95.7)1.06 (0.35–3.19).92311.9 (1.8–22.0)1.68 (1.15–2.45).0080.76 (0.33–1.19).001
    Decompression68.1 (46.5–89.7)3.27 (1.00–10.66).04910.1 (1.2–19.0)1.75 (1.20–2.55).0040.64 (0.21–1.06).004
    Radiotherapy73.5 (55.0–92.0)2.41 (0.74–7.86).1453.0 (0.0–8.2)3.39 (2.28–5.04)<.0011.37 (0.99–1.75)<.001
    Chemotherapy75.3 (52.0–98.5)1.01 (0.33–3.10).98613.8 (1.0–26.7)0.94 (0.64–1.39).7510.21 (−0.24–0.65).355
Treatment era
    1980–199678.0 (63.7–92.4)1.0051.8 (38.2–65.5)1.0024.9 (12.4–37.4)1.00−0.38 (−0.63 to –0.13).003
    1997–200488.7 (74.2–100.0)0.53 (0.14–2.00).52939.8 (16.9–62.6)1.01 (0.57–1.78).96820.5 (5.1–35.8)0.92 (0.59–1.45).728
    2005–201097.1 (91.5–100.0)0.53 (0.06–4.80).53453.8 (38.2–69.5)1.41 (0.77–2.61).26919.2 (1.4–36.9)1.27 (0.80–2.04).316
No. progressions1.43 (1.08–1.90).0121.16 (1.01–1.32).0300.40 (0.22–0.58)<.001
No. surgeries1.04 (0.96–1.14).3271.04 (1.01–1.08).0260.14 (0.08–0.19)<.001

Bold values indicate P < .05.

a

All patients with leptomeningeal metastases survived to last followup.

b

All patients with diencephalic syndrome and/ or leptomeningeal metastases progressed.

c

All patients aged <1 y at diagnosis, with diencephalic syndrome, hypothalamic involvement or leptomeningeal metastases experienced ≥1 endocrinopathy.

Table 2.

Twenty-Year OS, PFS, EEFS, and EMS at Last Followup With Crude HRs (95% CI) and Unadjusted Regression Coefficients β, Respectively

Independent VariableOS (95% CI)PFS (95% CI)EEFS (95% CI)EMS (95% CI)
20-Year OSCrude HRP20-Year PFSCrude HRP20-Year EEFSCrude HRPCrude βP
Age
    >5 y88.7 (76.1–100.0)1.0053.4 (36.1–70.8)1.0023.8 (13.2–34.5)1.00−0.02 (−0.07–0.04).600
    1–5 y80.0 (60.6–99.3)1.05 (0.28–3.94).94549.5 (34.4–64.5)1.17 (0.69–2.00).56625.6 (11.1–40.0)0.64 (0.43–0.97).034
    <1 y62.9 (32.6–93.1)5.29 (1.32–21.22).0196.7 (0.0–23.2)5.26 (2.66–10.42)<.0010.0c1.40 (0.78–2.53).260
Sex
    Male78.8 (55.7–100.0)1.0045.7 (27.5–63.8)1.0026.7 (14.2–39.2)1.00−0.11 (−0.54–0.32)
    Female82.6 (59.4–95.8)1.02 (0.34–3.03).97848.4 (36.8–60.1)1.37 (0.84–2.23).21116.5 (5.6–27.5)0.99 (0.68–1.45).972
Ethnicity (n = 150)
    White88.3 (79.6–96.9)1.0046.8 (34.2–59.4)1.0021.9 (11.7–32.0)1.000.01 (−0.53–0.55).979
    Nonwhite36.5 (0.0–89.5)2.43 (0.73–8.13).15137.3 (15.6–59.1)1.49 (0.85–2.62).16110.5 (0.0–27.4)1.24 (0.79–1.96).350
NF-1
    negative78.5 (63.4–93.7)1.0037.6 (24.9–50.3)1.0013.0 (4.7–21.3)1.00−0.96 (−1.37 to –0.55)<.001
    Positive88.2 (73.6–100.0)0.52 (0.14–1.90).52062.8 (45.0–80.7)0.43 (0.25–0.74).00233.0 (15.3–50.8)0.45 (0.30–0.68)<.001
Symptomatic
    No95.7 (87.6–100.0)1.0073.9 (56.0–91.9)1.0010.6 (0.0–37.4)1.001.00 (0.44–1.56).001
    Yes80.2 (67.3–93.1)1.53 (0.20–11.96).68644.0 (32.9–55.1)1.97 (0.85–4.56).11420.1 (11.8–28.4)1.93 (1.06–3.52).032
Diencephalic syndrome1.40 (0.63–2.17)<.001
    No80.8 (66.8–94.8)1.0053.3 (42.5–64.1)1.0023.5 (14.3–32.7)1.00
    Yes78.3 (50.8–100.0)1.99 (0.44–9.04).3750.0b4.13 (2.19–7.79)<.0010.0c1.74 (0.95–3.18).073
Tumor stage
    MDC 189.1 (74.2–100.0)1.0068.3 (33.1–100.0)1.0054.4 (21.0–87.8)1.000.27 (0.05–0.49).019
    MDC 282.8 (64.9–100.0)0.81 (0.16–4.23).80648.7 (34.3–63.2)3.64 (1.29–10.30).01513.5 (2.7–24.3)4.31 (2.05–9.08)<.001
    MDC 3/480.0 (55.2–100.0)0.71 (0.10–5.07).73537.3 (13.8–60.9)4.00 (1.34–11.97).01321.7 (6.4–37.0)3.45 (1.55–7.70).002
    MDC other midline80.4 (62.7–98.0)1.82 (0.33–10.03).49133.6 (12.7–54.6)4.93 (1.63–14.87).00514.1 (0.0–29.2)3.94 (1.74–8.91).001
Hypothalamic involvement1.11 (0.71–1.52)<.001
    No87.3 (76.4–98.3)1.0060.3 (46.4–74.3)1.0038.6 (25.9–51.3)1.00
    Yes73.1 (49.7–96.5)1.75 (0.59–5.20).31827.9 (12.5–43.3)2.86 (1.75–4.67)<.0010.0c3.03 (2.04–4.48)<.001
Leptomeningeal metastases1.46 (0.33–2.58).012
    No80.4 (67.4–93.3)1.0048.7 (37.8–59.5)1.0022.0 (13.3–30.7)1.00
    Yes100.0a0.05 (0.00-∞).6300.0b2.09 (0.76–5.78).1550.0c0.95 (0.35–2.59).922
Hydrocephalus
    No87.4 (86.8–87.1)1.0054.5 (41.0–68.0)1.0024.6 (12.6–36.5)1.000.47 (0.03–0.91).037
    Yes72.1 (47.5–96.8)2.00 (0.67–5.99).21335.0 (18.2–51.7)1.64 (1.01–2.66).04514.9 (3.7–26.2)1.65 (1.13–2.42).010
Tumor grade (n = 63)
    Grade I87.0 (76.7–94.7)1.0023.2 (6.93–39.5)1.0010.6 (0.0–21.4)1.000.30 (−0.73–1.34).559
    Grade II60.3 (10.6–100.0)2.71 (0.44–16.56).28035.9 (6.2–65.5)1.02 (0.45–2.32).9615.6 (0.0–20.0)1.86 (0.95–3.64).072
Primary treatment strategy
    Untreated91.9 (82.9–100.0)1.0059.3 (38.4–80.3)1.0033.3 (14.7–52.0)1.00
    Resection78.0 (55.6–100.0)0.70 (0.21–2.29).55342.7 (27.9–57.4)1.39 (0.85–2.27).18411.7 (1.0–22.5)1.76 (1.20–2.59).0040.66 (0.21–1.11).004
    Decompression68.7 (38.3–99.0)1.52 (0.50–4.65).46633.3 (15.5–51.1)1.61 (0.98–2.63).0609.5 (0.0–19.6)1.85 (1.25–2.74).0020.58 (0.12–1.04).014
    Radiotherapy71.8 (47.6–95.9)2.04 (0.68–6.11).20140.9 (21.2–60.6)1.05 (0.62–1.77).8654.1 (0.0–10.4)2.99 (2.00–4.46)<.0011.06 (0.60–1.51)<.001
    Chemotherapy84.7 (65.8–100.0)1.01 (0.28–3.67).98939.9 (22.6–57.2)1.64 (0.97–2.76).0639.1 (0.0–22.1)0.87 (0.56–1.33).515−0.13 (−0.62–0.36).603
Final treatment strategy
    Untreated100.0b1.0034.3 (8.2–60.4)1.00
    Resection76.5 (57.3–95.7)1.06 (0.35–3.19).92311.9 (1.8–22.0)1.68 (1.15–2.45).0080.76 (0.33–1.19).001
    Decompression68.1 (46.5–89.7)3.27 (1.00–10.66).04910.1 (1.2–19.0)1.75 (1.20–2.55).0040.64 (0.21–1.06).004
    Radiotherapy73.5 (55.0–92.0)2.41 (0.74–7.86).1453.0 (0.0–8.2)3.39 (2.28–5.04)<.0011.37 (0.99–1.75)<.001
    Chemotherapy75.3 (52.0–98.5)1.01 (0.33–3.10).98613.8 (1.0–26.7)0.94 (0.64–1.39).7510.21 (−0.24–0.65).355
Treatment era
    1980–199678.0 (63.7–92.4)1.0051.8 (38.2–65.5)1.0024.9 (12.4–37.4)1.00−0.38 (−0.63 to –0.13).003
    1997–200488.7 (74.2–100.0)0.53 (0.14–2.00).52939.8 (16.9–62.6)1.01 (0.57–1.78).96820.5 (5.1–35.8)0.92 (0.59–1.45).728
    2005–201097.1 (91.5–100.0)0.53 (0.06–4.80).53453.8 (38.2–69.5)1.41 (0.77–2.61).26919.2 (1.4–36.9)1.27 (0.80–2.04).316
No. progressions1.43 (1.08–1.90).0121.16 (1.01–1.32).0300.40 (0.22–0.58)<.001
No. surgeries1.04 (0.96–1.14).3271.04 (1.01–1.08).0260.14 (0.08–0.19)<.001
Independent VariableOS (95% CI)PFS (95% CI)EEFS (95% CI)EMS (95% CI)
20-Year OSCrude HRP20-Year PFSCrude HRP20-Year EEFSCrude HRPCrude βP
Age
    >5 y88.7 (76.1–100.0)1.0053.4 (36.1–70.8)1.0023.8 (13.2–34.5)1.00−0.02 (−0.07–0.04).600
    1–5 y80.0 (60.6–99.3)1.05 (0.28–3.94).94549.5 (34.4–64.5)1.17 (0.69–2.00).56625.6 (11.1–40.0)0.64 (0.43–0.97).034
    <1 y62.9 (32.6–93.1)5.29 (1.32–21.22).0196.7 (0.0–23.2)5.26 (2.66–10.42)<.0010.0c1.40 (0.78–2.53).260
Sex
    Male78.8 (55.7–100.0)1.0045.7 (27.5–63.8)1.0026.7 (14.2–39.2)1.00−0.11 (−0.54–0.32)
    Female82.6 (59.4–95.8)1.02 (0.34–3.03).97848.4 (36.8–60.1)1.37 (0.84–2.23).21116.5 (5.6–27.5)0.99 (0.68–1.45).972
Ethnicity (n = 150)
    White88.3 (79.6–96.9)1.0046.8 (34.2–59.4)1.0021.9 (11.7–32.0)1.000.01 (−0.53–0.55).979
    Nonwhite36.5 (0.0–89.5)2.43 (0.73–8.13).15137.3 (15.6–59.1)1.49 (0.85–2.62).16110.5 (0.0–27.4)1.24 (0.79–1.96).350
NF-1
    negative78.5 (63.4–93.7)1.0037.6 (24.9–50.3)1.0013.0 (4.7–21.3)1.00−0.96 (−1.37 to –0.55)<.001
    Positive88.2 (73.6–100.0)0.52 (0.14–1.90).52062.8 (45.0–80.7)0.43 (0.25–0.74).00233.0 (15.3–50.8)0.45 (0.30–0.68)<.001
Symptomatic
    No95.7 (87.6–100.0)1.0073.9 (56.0–91.9)1.0010.6 (0.0–37.4)1.001.00 (0.44–1.56).001
    Yes80.2 (67.3–93.1)1.53 (0.20–11.96).68644.0 (32.9–55.1)1.97 (0.85–4.56).11420.1 (11.8–28.4)1.93 (1.06–3.52).032
Diencephalic syndrome1.40 (0.63–2.17)<.001
    No80.8 (66.8–94.8)1.0053.3 (42.5–64.1)1.0023.5 (14.3–32.7)1.00
    Yes78.3 (50.8–100.0)1.99 (0.44–9.04).3750.0b4.13 (2.19–7.79)<.0010.0c1.74 (0.95–3.18).073
Tumor stage
    MDC 189.1 (74.2–100.0)1.0068.3 (33.1–100.0)1.0054.4 (21.0–87.8)1.000.27 (0.05–0.49).019
    MDC 282.8 (64.9–100.0)0.81 (0.16–4.23).80648.7 (34.3–63.2)3.64 (1.29–10.30).01513.5 (2.7–24.3)4.31 (2.05–9.08)<.001
    MDC 3/480.0 (55.2–100.0)0.71 (0.10–5.07).73537.3 (13.8–60.9)4.00 (1.34–11.97).01321.7 (6.4–37.0)3.45 (1.55–7.70).002
    MDC other midline80.4 (62.7–98.0)1.82 (0.33–10.03).49133.6 (12.7–54.6)4.93 (1.63–14.87).00514.1 (0.0–29.2)3.94 (1.74–8.91).001
Hypothalamic involvement1.11 (0.71–1.52)<.001
    No87.3 (76.4–98.3)1.0060.3 (46.4–74.3)1.0038.6 (25.9–51.3)1.00
    Yes73.1 (49.7–96.5)1.75 (0.59–5.20).31827.9 (12.5–43.3)2.86 (1.75–4.67)<.0010.0c3.03 (2.04–4.48)<.001
Leptomeningeal metastases1.46 (0.33–2.58).012
    No80.4 (67.4–93.3)1.0048.7 (37.8–59.5)1.0022.0 (13.3–30.7)1.00
    Yes100.0a0.05 (0.00-∞).6300.0b2.09 (0.76–5.78).1550.0c0.95 (0.35–2.59).922
Hydrocephalus
    No87.4 (86.8–87.1)1.0054.5 (41.0–68.0)1.0024.6 (12.6–36.5)1.000.47 (0.03–0.91).037
    Yes72.1 (47.5–96.8)2.00 (0.67–5.99).21335.0 (18.2–51.7)1.64 (1.01–2.66).04514.9 (3.7–26.2)1.65 (1.13–2.42).010
Tumor grade (n = 63)
    Grade I87.0 (76.7–94.7)1.0023.2 (6.93–39.5)1.0010.6 (0.0–21.4)1.000.30 (−0.73–1.34).559
    Grade II60.3 (10.6–100.0)2.71 (0.44–16.56).28035.9 (6.2–65.5)1.02 (0.45–2.32).9615.6 (0.0–20.0)1.86 (0.95–3.64).072
Primary treatment strategy
    Untreated91.9 (82.9–100.0)1.0059.3 (38.4–80.3)1.0033.3 (14.7–52.0)1.00
    Resection78.0 (55.6–100.0)0.70 (0.21–2.29).55342.7 (27.9–57.4)1.39 (0.85–2.27).18411.7 (1.0–22.5)1.76 (1.20–2.59).0040.66 (0.21–1.11).004
    Decompression68.7 (38.3–99.0)1.52 (0.50–4.65).46633.3 (15.5–51.1)1.61 (0.98–2.63).0609.5 (0.0–19.6)1.85 (1.25–2.74).0020.58 (0.12–1.04).014
    Radiotherapy71.8 (47.6–95.9)2.04 (0.68–6.11).20140.9 (21.2–60.6)1.05 (0.62–1.77).8654.1 (0.0–10.4)2.99 (2.00–4.46)<.0011.06 (0.60–1.51)<.001
    Chemotherapy84.7 (65.8–100.0)1.01 (0.28–3.67).98939.9 (22.6–57.2)1.64 (0.97–2.76).0639.1 (0.0–22.1)0.87 (0.56–1.33).515−0.13 (−0.62–0.36).603
Final treatment strategy
    Untreated100.0b1.0034.3 (8.2–60.4)1.00
    Resection76.5 (57.3–95.7)1.06 (0.35–3.19).92311.9 (1.8–22.0)1.68 (1.15–2.45).0080.76 (0.33–1.19).001
    Decompression68.1 (46.5–89.7)3.27 (1.00–10.66).04910.1 (1.2–19.0)1.75 (1.20–2.55).0040.64 (0.21–1.06).004
    Radiotherapy73.5 (55.0–92.0)2.41 (0.74–7.86).1453.0 (0.0–8.2)3.39 (2.28–5.04)<.0011.37 (0.99–1.75)<.001
    Chemotherapy75.3 (52.0–98.5)1.01 (0.33–3.10).98613.8 (1.0–26.7)0.94 (0.64–1.39).7510.21 (−0.24–0.65).355
Treatment era
    1980–199678.0 (63.7–92.4)1.0051.8 (38.2–65.5)1.0024.9 (12.4–37.4)1.00−0.38 (−0.63 to –0.13).003
    1997–200488.7 (74.2–100.0)0.53 (0.14–2.00).52939.8 (16.9–62.6)1.01 (0.57–1.78).96820.5 (5.1–35.8)0.92 (0.59–1.45).728
    2005–201097.1 (91.5–100.0)0.53 (0.06–4.80).53453.8 (38.2–69.5)1.41 (0.77–2.61).26919.2 (1.4–36.9)1.27 (0.80–2.04).316
No. progressions1.43 (1.08–1.90).0121.16 (1.01–1.32).0300.40 (0.22–0.58)<.001
No. surgeries1.04 (0.96–1.14).3271.04 (1.01–1.08).0260.14 (0.08–0.19)<.001

Bold values indicate P < .05.

a

All patients with leptomeningeal metastases survived to last followup.

b

All patients with diencephalic syndrome and/ or leptomeningeal metastases progressed.

c

All patients aged <1 y at diagnosis, with diencephalic syndrome, hypothalamic involvement or leptomeningeal metastases experienced ≥1 endocrinopathy.

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