Table 1 Presenting features of 268 stage 4 s neuroblastoma patients
Feature | All patients | Treatment era | p | ||
|---|---|---|---|---|---|
1979–1984 | 1985–1999 | 2000–2013 | |||
No. (%) | No. (%) | No. (%) | No. (%) | ||
268 (100) | 26 (9.7) | 116 (43.3) | 126 (47.0) | ||
Demographic and clinical features | |||||
Gender | |||||
Male | 156 (58.2) | 17 (65.4) | 70 (60.3) | 69 (54.8) | 0.0501 |
Female | 112 (41.8) | 9 (34.6) | 46 (39.7) | 57 (45.2) | |
Age, days. Median (IQR) | 87 (33.5–146.5) | 75.5 (34–122) | 77 (22.5–141.5) | 97.5 (37–170) | |
Age, days | |||||
0–29 | 60 (22.4) | 6 (23.1) | 31 (26.7) | 23 (18.3) | 0.619 |
30–59 | 40 (14.9) | 4 (15.4) | 14 (12.1) | 22 (17.5) | |
60–89 | 37 (13.8) | 6 (23.1) | 15 (12.9) | 16 (12.7) | |
90–119 | 40 (14.9) | 3 (11.5) | 19 (16.4) | 18 (14.3) | |
120–365 | 91 (33.9) | 7 (26.9) | 37 (31.9) | 47 (37.3) | |
Age, days | |||||
0–29 | 60 (22.4) | 6 (23.1) | 31 (26.7) | 23 (18.3) | 0.286 |
30–365 | 208 (77.6) | 20 (76.9) | 85 (73.3) | 103 (81.7) | |
Symptoms at presentation# | |||||
None | 17 (6.3) | 0 | 4 (3.5) | 13 (10.3) | 0.039* |
Yes, minor | 51 (19.0) | 3 (11.5) | 19 (16.4) | 29 (23.0) | |
Yes, major | 200 (74.6) | 23 (88.5) | 93 (80.2) | 84 (66.7) | |
Major symptoms | 200 (74.6) | 23 (88.5) | 93 (80.2) | 84 (66.7) | |
Hepatomegaly, yes | 186 (69.4) | 21 (80.8) | 85 (73.3) | 80 (63.5) | 0.107 |
Dyspnea, yes | 52 (19.4) | 4 (15.4) | 25 (21.5) | 23 (18.2) | 0.699 |
Organ dysfunction, yes | 34 (12.7) | 3 (11.5) | 9 (7.8) | 22 (17.5) | 0.073* |
Combinations of major symptoms | |||||
No major symptoms or no symptom | 68 (25.4) | 3 (11.5) | 23 (19.8) | 42 (33.3) | 0.033* |
Organ dysfunction only | 2 (0.8) | 1 (3.9) | 0 | 1 (0.8) | |
Dyspnea ± Organ dysfunction | 12 (4.5) | 1 (3.9) | 8 (6.9) | 3 (2.4) | |
Hepatomegaly ± Organ dysfunction | 146 (54.5) | 18 (69.2) | 68 (58.6) | 60 (47.6) | |
Hepatomegaly + Dyspnea (± Organ dysfunction) | 40 (14.9) | 3 (11.5) | 17 (14.7) | 20 (15.9) | |
Minor symptoms | |||||
Skin nodules, yes | 42 (15.7) | 4 (15.4) | 28 (24.1) | 10 (7.9) | 0.002* |
Abdominal mass, yes | 34 (12.7) | 1 (3.8) | 12 (10.3) | 21 (16.7) | 0.139* |
Cervical mass, yes | 11 (4.1) | 0 | 5 (4.3) | 6 (4.8) | 0.814* |
Neurologic symptoms, yes | 12 (4.5) | 0 | 6 (5.2) | 6 (4.8) | 0.753* |
Primary site | |||||
Adrenal^ | 175 (65.3) | 16 (61.5) | 77 (66.4) | 82 (65.1) | 0.272* |
Retroperitoneal ganglia | 49 (18.3) | 4 (15.4) | 16 (13.8) | 29 (23.0) | |
Thorax | 22 (8.2) | 3 (11.5) | 11 (9.5) | 8 (6.3) | |
Neck | 9 (3.4) | 0 | 5 (4.3) | 4 (3.2) | |
Not identified | 13 (4.9) | 3 (11.5) | 7 (6.0) | 3 (2.4) | |
Liver infiltration, yes | |||||
yes | 230 (85.8) | 22 (84.6) | 102 (87.9) | 106 (84.1) | 0.689* |
Positive bone marrow cytology, yes | |||||
yes | 111 (41.4) | 8 (30.8) | 42 (36.2) | 61 (48.4) | 0.082* |
Biochemical, biologic and histologic features | |||||
Urine VMA (222 tested) | |||||
Normal | 59 (26.6) | 2 (8.3) | 22 (22.9) | 35 (34.3) | 0.019 |
Elevated | 163 (73.4) | 22 (91.7) | 74 (77.1) | 67 (65.7) | |
Urine HVA (112 tested) | |||||
Normal | 18 (16.1) | 0 | 2 (6.1) | 16 (21.0) | 0.149* |
Elevated | 94 (83.9) | 3 (100) | 31 (93.9) | 60 (79.0) | |
Serum LDH (227 tested) | |||||
Normal | 131 (57.7) | 7 (87.5) | 78 (75.7) | 46 (39.7) | < 0.001* |
Elevated | 96 (42.3) | 1 (12.5) | 25 (24.3) | 70 (60.3) | |
Serum ferritin (193 tested) | |||||
Normal | 116 (60.1) | 5 (100) | 55 (61.1) | 56 (57.1) | 0.176* |
Elevated | 77 (39.9) | 0 | 35 (38.9) | 42 (42.9) | |
MYCN gene (183 tested) | |||||
Normal | 168 (91.8) | 0 | 61 (91.0) | 107 (92.2) | 0.776 |
Amplified | 15 (8.2) | 0 | 6 (9.0) | 9 (7.8) | |
1p chromosome (138 tested) | |||||
Normal | 110 (79.7) | 1 (100) | 32 (76.2) | 77 (81.1) | 0.603* |
Deleted | 28 (20.3) | 0 | 10 (23.8) | 18 (18.9) | |
DNA index (121 tested) | |||||
Aneuploid | 80 (66.1) | 0 | 24 (60.0) | 56 (69.1) | 0.318 |
Di-tetraploid | 41 (33.9) | 0 | 16 (40.0) | 25 (30.9) | |
Histology by INPC (75 tested) | |||||
Favorable | 69 (92.0) | 0 | 0 | 69 (92.0) | – |
Unfavorable | 6 (8.0) | 0 | 0 | 6 (8.0) | |