Campbell K, Kao PC, Naranjo A, Kamijo T, Ramanujachar R, London WB, DuBois SG. Clinical and biological features prognostic of survival after relapse or progression of INRGSS stage MS pattern neuroblastoma: A report from the International Neuroblastoma Risk Group (INRG) project. Pediatr Blood Cancer. 2023 Feb;70(2):e30054. doi: 10.1002/pbc.30054. Epub 2022 Oct 31. PubMed PMID: 36316811; PubMed Central PMCID: PMC9988328.
Publication Citation
Abstract
Outcomes for patients with INRGSS MS metastatic pattern neuroblastoma at initial diagnosis are well-described. Prognosis after an initial event (relapse, progression, secondary malignancy) is unclear. We investigated characteristics of MS pattern neuroblastoma patients in the International Neuroblastoma Risk Group database who subsequently experienced an event. Post-event overall survival (OS) ± standard error was calculated overall and by diagnosis era: <2000 vs ≥2001. Cox models were used to identify factors prognostic of post-event OS. Among 209 patients with an event, 88% were <365 days old at diagnosis; tumors were MYCN amplified in 24% and diploid in 33%. The median (range) time from diagnosis to first event was 8.16 months (7 days-11.24 years). Of 96 patients with known relapse/progression pattern, 75% were metastatic or primary plus metastatic. Five-year post-event OS was 53±3.6% and was higher for ≥2001 (62±5.0%) compared to <2001 (44±4.9%; p=0.0046). In patients diagnosed ≥2001, older age, Hispanic ethnicity, MYCN amplification, 1p LOH, diploidy, high MKI, high LDH, unfavorable histology, and shorter time to first event were prognostic of worse post-event OS. Independent adverse prognostic factors on multivariable testing were non-White race, MYCN amplification, and diploidy. Patients diagnosed ≥2001 have substantially better post-event OS compared to <2001. In those diagnosed ≥2001, most well-accepted prognostic factors for OS at diagnosis are also prognostic of post-event OS. Future studies may evaluate strategies to improve outcomes in this rare population.