Table 3.
Age <30 Years
|
Age >30 Years
|
|||
---|---|---|---|---|
Group | NF1 with MPNSTs | NF1 without MPNSTs | NF1 with MPNSTs | NF1 without MPNSTs |
Total number | 6 | 11 | 7 | 15 |
Median number of externally visible plexiform neurofibromas (range) | 0.5 (0–3) | 1 (0–3) | 1 (0–10) | 1 (0–5) |
Median number of subcutaneous neurofibromas (range) | 112.5* (0–2000) | 2* (0–30) | 40 (10–300) | 25 (0–1000) |
Median number of cutaneous neurofibromas (range) | 0 (0–400) | 4 (0–300) | 300 (0–5000) | 30 (0–1000) |
Number of patients with internal neurofibromas on MRI (%) | 6‡ (100) | 3‡ (27) | 3 (43) | 10 (67) |
Median number of internal neurofibromas >3 cm (range) | 1† (1–4) | 0† (0–3) | 0 (0–3) | 1 (0–9) |
Median internal neurofibroma volume, ml (range) | 460** (8–2252) | 0** (0–5588) | 0 (0–825) | 115 (0–7030) |
Number with spinal neurofibromas (%) | 4 (67) | 6 (54) | 5 (71) | 6 (40) |
Abbreviations: NF1, neurofibromatosis 1; MPNSTs, malignant peripheral nerve sheath tumors.
p = 0.048 by Mann-Whitney U test;
p = 0.027 by Mann-Whitney U test;
p = 0.009 by Fisher’s exact test;
p = 0.037 by Mann-Whitney U test.