Adverse outcome of infants with metastatic neuroblastoma, MYCN amplification and/or bone lesions: results of the French Society of Pediatric Oncology

Abstract

To assess the relevance of MYCN amplification and bone lesions in stage 4 neuroblastoma (NB) in infants aged <1 year, 51 infants with stage 4 NB were enrolled. Three groups of patients were defined according to the type of metastases and the resectability of the primary tumour. Group I comprised 21 infants with radiologically detectable bone lesions, Group II 22 patients with an unresectable primary tumour and Group III eight patients with only metaiodobenzylguanidine (MIBG) skeletal uptake. MYCN oncogene content was assayed in 47/51 tumours and found to be amplified in 17 (37%). The 5-year event-free survival (EFS) rate of these 51 infants was 64.1% (± 7.1%). In a univariate analysis, bone lesions, MYCN amplification, urinary vanillylmandelic/homovanillic acid ratio and serum ferritin levels adversely influenced outcome. In the multivariate analysis, radiologically detectable bone lesions were the most powerful unfavourable prognostic indicator: the EFS rate was 27.2% for these infants compared to 90% for infants without bone lesions (P < 0.0001). Our data emphasize the poor prognosis of infants affected by stage 4 NB with bone lesions, especially when associated with MYCN amplification. Given the poor results in this group whatever the treatment, new therapeutic approaches need to be investigated in the future. © 2000 Cancer Research Campaign

Localized cervical neuroblastoma: prevention of surgical complications

“…The primary tumor was staged according to International Neuroblastoma Staging System (INSS). N-myc amplification was performed in all cases and results showing 10 or more copies per haploid genome were considered as a highly unfavorable factor [8][9][10].…”

“…The intensity of treatment was markedly lower for all patients except those with N-myc amplification than on the first study (NBL90) ( Table 2) [8,11]. All operative reports were reviewed to evaluate the extent of resection and amount of residual tumor.…”

“…Between March 1990 and September 1999, all cases of localized neuroblastoma treated at 31 centers of The French Society of Pediatric Oncology (SFOP) were included in two consecutive prospective multicenter studies. In the first study (NBL90) [7][8][9][10], patients with localized NB were treated by primary surgery if complete gross resection was considered as feasible without mortality or major functional morbidity but no specific criteria of resectability were used to select patients. Overall in the first study (i.e.…”

Expression of p53, or targeting towards EGFR, enhances the oncolytic potency of conditionally replicative adenovirus against neuroblastoma

“…Although the outcome of infants and older children with local or regional disease has improved significantly, little improvement has occurred in the outlook for older children with metastatic disease at diagnosis. Moreover, children with tumors with amplified MYCN gene do badly, independent of age and tumor stage [1,2]. Despite intense chemo-radiotherapy supported with autologous stem cell rescue, 60-70% of high-risk NB patients develop recurrent disease that is refractory to additional therapy [3].…”

The Buried Vertical Mattress: A Simplified Technique for Eyelid Margin Repair

“…3 Prognostic indicators helpful in predicting outcomes in patients with neuroblastic tumors include age at diagnosis, 8,9 stage of disease, 10,11 DI of tumor, 8,12 MYCN amplification, [11][12][13] primary site, 8,9 and histopathologic subtype of tumor. 14 Patients younger than 1 year of age with neuroblastic tumors have a better prognosis than those older than 1 year.…”