| Dombi et al25
|
US longitudinal study to analyze changes in PN volume in relation to age and body growth in children and young adults with NF1 and inoperable, symptomatic, or progressive PNs, using a sensitive, automated method of volumetric MRI analysis |
N = 49
Median age: 8.3 years |
Median: 34 months |
Included patients had a total of 61 PNs 69% of patients experienced an increase of ≥20% of PN volume PN volume increased more rapidly than body weight over time (P = 0.026) Younger patients had the most rapid PN growth rate |
| Nguyen et al26
|
German retrospective study to assess internal PNs using volumetric WBMRI |
N = 171
Median age: 28.6 years |
Median 2.2 years |
71 of the 171 included patients had a total of 200 PNs Median growth rate in whole-body tumor volume was 3.7% per year This growth rate correlated with larger whole-body tumor volume (P < 0.001) and younger age (P = 0.004) 13.5% of individual tumors increased in size by >20% per year, on average; these were significantly more frequent among children than among adults (P < 0.001) 70% of these tumors were found in pediatric patients, representing 29% of tumors found in the pediatric age group 30% of these tumors were found in those aged >18 years, representing 6.0% of tumors found in this age group |
| Gross et al11
|
Retrospective review of patients enrolled in the National Cancer Institute Natural History Study of Patients with Neurofibromatosis Type 1 (NCT00924196) using volumetric MRI analysis |
N = 41
Median age: 8 years |
At least yearly until age 18, then every 1–3 years |
Included patients had a total of 57 PNs 86% of PNs had >20% increase in tumor volume from baseline to maximum assessment Median 108.9% change in PN volume between baseline and maximum assessment Observed 15.9% per year median PN growth rate Median PN growth rate was 35.1% per year in patients aged 3–5 years and 13.1% per year for patients aged 11–25 years Patients with NF1 and PN who reported increased use of pain medication experienced 21% growth in PNs per year (vs 13% growth in PNs per year in patients with NF1 and PN who did not report increased use of pain medication) |
| Tucker et al27
|
German study to characterize the growth of PNs in patients with NF1 between 1990 and 2006 using serial MRIs |
N = 34
Mean age: 10 years |
Median: 6.0 years |
Included patients had a total of 44 PNs 14 PNs were symptomatic (4 were superficial and displacing, 3 were superficial and invasive, 2 were deep and displacing, and 5 were deep and invasive) Superficial tumors grew significantly more quickly than deep tumors (P = 0.034) The growth rate of tumors was significantly greater in patients aged <10 years at initial examination than in patients aged >10 years at initial examination (0.7 cm2/year vs 0.03 cm2/year; P = 0.014) 30% of the PNs identified in patients aged <10 years (n = 18) were symptomatic, which included 4 of 9 (44%) rapidly progressing tumors |
| Dagalakis et al28
|
Retrospective study using data from the National Cancer Institute NF1 Natural History Study to evaluate the relationship between pubertal progression and change in PN burden over time in pediatric and young adult patients with NF1 and PN |
N = 41
Actively progressing through puberty (n = 16); age range: 7–20 years
Peripubertal (n = 25); age range: 6–11 years and 17–33 years |
Mean: 20 months |
The number of PNs was not stated No statistically significant differences in tumor burden change over time between the groups (P = 0.31) No statistically significant differences in peripubertal and pubertal PN growth rates (P = 0.90) |
