Table 1.
Patient and tumor characteristics for PNs and DNLs#
| Plexiform Neurofibroma | Distinct Nodular Lesion | |||||
|---|---|---|---|---|---|---|
| Median (min, max) 10th, 90th Percentile | Entire Follow-Up N = 113 | Treatment-Free Follow-Up N = 70 | No Prior Treatment# N = 42 | Entire Follow-Up N = 79 | Treatment-Free Follow-Up N = 61 | No Prior Treatment# N = 24 |
| Age at initial MRI, y | 8.4 (0.7, 40.2) 3.2, 18.7 | 10.3 (0.7, 40.2) 3.4, 20.5 | 8.2 (0.7, 40.2) 1.8, 20.2 | 15.2 (2.2, 45.3) 7.2, 25.7 | 18.1 (3.7, 40.2) 8.7, 25.7 | 18.9 (3.7, 40.2) 5.2, 30.5 |
| Initial tumor volume, mL | 295 (4, 4895) 29, 1837 | 374 (4, 4895) 27, 1862 | 273 (4, 4895) 13, 1808 | 13 (2, 122) 5, 66 | 17 (2, 201) 6, 79 | 20 (2, 122) 5, 80 |
| Final tumor volume, mL | 606 (14, 6980) 47, 2990 | 563 (14, 6513) 41, 2210 | 388 (14, 6513) 36, 1970 | 37 (9, 345) 12, 129 | 37 (9, 345) 13, 130 | 35 (11, 172) 15, 139 |
| Duration of follow-up, y | 5.6 (1.0, 14.3) 2.2, 11.1 | 3.0 (1.0, 12.9) 1.2, 7.7 | 2.7 (1.0, 12.4) 1.2, 7.7 | 4.2 (1.0, 12.9) 2.1, 7.7 | 3.7 (1.0, 12.9) 1.5, 6.3 | 3.3 (1.1, 8.0) 1.4, 5.5 |
| # MRI/tumor | 10 (2, 35) 4, 18 | 5 (3, 14) 3, 8 | 5 (3, 14) 3, 8 | 5 (2, 17) 3, 10 | 4 (3, 12) 3, 7 | 4 (3, 9) 3, 6 |
| Growth rate (%/, y)* | NA | 12.4 (−8.5, 246.7) −1.7, 47.9 | 16.6 (−5.3, 246.7) 0.6, 101.7 | NA | 27.1 (−11.9, 290.8) 2.7, 97.5 | 28.1 (1.1, 117.2) 4.8, 88.8 |
| Tumor volume % change final-initial volume per year (%/y) (%/,y)** | 13.9 (−5.8, 252.0) 0.5, 51.3 | 31.5 (−2.2, 343.5) 2.9, 121.3 | ||||
#During the entire period of follow-up, the first treatment-free portion of follow-up during which growth rate was estimated, and the subset where first treatment-free period was before patient received any therapies. No prior treatment group includes a subset of the “treatment-free” group where the patients had not received any tumor-directed medical therapies prior to the “treatment-free” portion of follow-up for which growth rate was calculated.
*Slope estimates from linear regression were used to estimate growth rate during the treatment-free portion of follow-up.
**Tumor volume % change final-initial volume per year (%/y) = 100 x (Final tumor volume − initial tumor volume)/initial tumor volume divided by the duration of follow-up in years during the entire period of follow-up.