PATHOLOGY REVIEW OF SCREENING NEGATIVE NEUROBLASTOMAS - A REPORT FROMTHE QUEBEC-NEUROBLASTOMA-SCREENING-PROJECT

BACKGROUND. The Quebec Neuroblastoma Screening (QNS) Project completed a 5-year program for measuring urinary vanillylmandelic acid (VMA)/ho movanillic acid (HVA) levels at age 3 weeks and/or 6 months in 89% of 476,603 Quebec-born infants from 1989-1994; 45 screening positive prec linical cases (S-positive cases) and 20 congenital/neonatal (C/N) case s were identified. As of April 1997, an additional 59 cases in the sam e birth cohort were diagnosed clinically; these neuroblastomas develop ed after screening verified normal VMA/HVA levels (S-negative cases). METHODS. Pathology specimens from 45 of 59 S-negative cases were revie wed centrally and classified according to the Shimada system. Results were compared with clinical data and also with S-positive and C/N case s. RESULTS, Of 45 S-negative cases, 27 tumors had favorable histology (FH) and 18 had unfavorable histology (UH). Approximately 52% of FH tu mors were diagnosed before age 1 year, whereas UH tumors were nearly e xclusively (94%) diagnosed after age 1 year (P < 0.01). Approximately 89% of FH tumors were Stage I, II, or IV-S, whereas 72% UH tumors were Stage III or IV (P < 0.001).All children with FH tumors were alive at last follow-up (range of follow-up period: 9-79 months; median, 35 mo nths), whereas 8 children with Un tumors died of disease even after li mited follow-up (range of follow-up period: 0-60 months; median, 20 mo nths). By contrast, S-positive and C/N cases were predominantly (37%) FH rumors, often (76%) Stage I, II, or IV-S, with excellent clinical o utcome (survival rate of 98%). CONCLUSIONS, The majority of the UH neu roblastomas that developed in the birth cohort of the QNS Project were included in the group of S-negative cases and could not be detected b y the screening at age 3 weeks and/or 6 months. Cancer 1998;83: 575-81 , (C) 1998 American Cancer Society.