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Background: Patients with metastatic NB typically have a poor prognosis; however case series have suggested that those with 4N disease may have improved outcomes. Methods: Retrospective analysis of data from INRG database for patients diagnosed 1990–2002. 4N patients (INSS stage 4 disease confined to distant lymph nodes) were compared to the balance of stage 4 patients (‘non-4N’), excluding those with missing metastatic site data. 5-yr estimates of overall (OS) and event-free survival (EFS) were calculated ± standard error (Kaplan-Meier method). Patient characteristics were compared by Mann-Whitney or Fisher’s exact/Chi-square tests. Results: 2,250 INSS stage 4 patients with complete data were identified, of whom 146 (6%) had 4N disease. For 4N patients, EFS and OS (5-yr: 77% ± 4%, 85% ± 3%), were significantly better than EFS and OS (5-yr: 35% ± 1%, 42% ± 1%) for non-4N stage 4 patients (p<0.0001). 4N patients were more likely to be younger (median age at diagnosis 1.2 yr vs 2.5 yr for non-4N; p<0.0001) and have tumors with favourable International Neuroblastoma Pathologic Classification (INPC) (63% vs 26%, p<0.0001), differentiating grade (21% vs 8%, p=0.006), lower MKI (p=0.0011) and non-MYCN amplified tumors (89% vs 69%, p<0.0001). Within subgroups defined by age at diagnosis and MYCN status, 4N pattern of disease remained significantly associated with improved outcomes. For patients aged 547 days at diagnosis and MYCN non-amplified, 5-yr EFS for 4N patients (n=42) was 63% ± 8% vs 27% ± 2% for non-4N (n=785); OS 74% ± 7% vs 38% ± 2% (both p<0.0001). Within this subgroup, favourable INPC and differentiating grade remained more frequent in the 4N vs non-4N patients (45% vs 10%, p<0.0001; 45% vs 8%, p=0.0017, respectively). Conclusions: 4N represents a subgroup of metastatic patients with better outcome than other INSS stage 4 patients. These findings indicate that the biology and response to treatment of 4N tumors differs from other stage 4 tumors, and different therapies should be considered for this cohort. Future exploration of biological factors determining pattern of metastatic spread and response to therapy is warranted.

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