Indolent course of advanced neuroblastoma in children older than 6 years at diagnosis

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Abstract
Background. An early observation suggests that children older than 6 years of age at diagnosis of neuroblastoma constitute a favorable prognostic group. Methods. Kaplan-Meier plots of survival of all such patients diagnosed at the Children's Hospital of Pittsburgh 1975–1992 were compared with curves of concurrently treated patients with Stage IV disease who were 1-6 years of age at diagnosis (“younger patients”). Known prognostic features, including stage and primary site of disease, pattern of metastases, histopathology, MYCN gene amplification, and urinary catecholamine metabolite ratios, were reviewed. Results. Of 17 children diagnosed after the age of 6 years (“older patients”), 13 patients had Evans' Stage IV disease and 4 had Stage III disease. The median survival was 3.24 years (range, 0.63–15.04 years) for the entire cohort and 3.07 years for those children with Stage IV disease. This compared with a median survival of 1.05 years in 34 concurrent younger patients (P < 0.01). In most cases, disease in these older patients was characterized by a short-lived complete or partial remission followed by aggressive recurrent disease that was partially and only transiently chemo- or radiosensitive. Only 3 patients (2 with Stage IV disease) are in continuous complete remission at 3, 510/12, and 141/2 years from diagnosis. Although poor prognostic factors were common, including the presence of bony metastases (12/17), biopsy material from pretreatment tumor specimens demonstrated a single MYCN gene copy number in all patients and favorable histology in 15 of 16 samples. Conclusion. Older children with neuroblastoma have a more indolent course than do younger patients, a finding that appears to be related to favorable histology and the absence of MYCN amplification. Examination of larger numbers of such patients from cooperative groups should lead to a better understanding of what appears to be a subset of pediatric patients with neuroblastoma who may benefit from specifically tailored treatment protoc.