The impact of gross total resection on local control and survival in high-risk neuroblastoma

Presented at the 34th Annual Meeting of the American Pediatric Surgical Association, Fort Lauderdale, Florida, May 25–28, 2003.
https://doi.org/10.1016/j.jpedsurg.2003.11.028Get rights and content

Abstract

Background/purpose

Gross total resection of the primary tumor in treatment of high-risk neuroblastoma remains controversial. Furthermore, there are few reports of the effect of primary tumor resection on local control as opposed to overall survival. The authors reviewed their institutional experience to assess the effect of primary tumor resection on local control and overall survival.

Methods

A total of 141 patients were treated on protocol between November 1, 1979 and June 25, 2002 and are the subject of this report. Gross total resection was assessed by review of operative notes, postoperative computerized axial tomograms, and postoperative meta-iodobenzyl guanidine (MIBG)1 scans when available.

Results

The median age was 3.3 years, and all patients were International Neuroblastoma Staging System (INSS) stage 4 with 79% having metastases to cortical bone. The primary site was the adrenal gland in 74%, the central abdominal compartment in 13%, the posterior mediastinum in 7%, and other sites in 6%. Gross total resection was accomplished in 103 (73%) but was more than 90% for the last 3 protocols. Five kidneys were lost overall. The probability of local progression was 50% in unresected patients compared with 10% in patients undergoing gross total resection (P < .01). Overall survival rate in resected patients was 50% compared with 11% in unresected patients (P < .01).

Conclusions

Our data indicate that local control and overall survival rate are correlated with gross total resection of the primary tumor in high-risk neuroblastoma. Gross total resection should be part of the management of stage 4 neuroblastoma in patients greater than 1 year of age.

Section snippets

Materials and methods

Institutional review board permission was obtained. Criteria for entry into this retrospective study included age of 1 year or older at diagnosis and stage 4 disease by INSS criteria. All patients received the majority of their treatment at Memorial Sloan-Kettering Cancer Center. Patient data were obtained from operative records and the pediatric and neuroblastoma databases. Our center evaluates and treats 70 to 90 neuroblastoma patients per year. From this cohort there were 143 consecutive

Patient characteristics

The median age at diagnosis was 3.3 years and ranged from 1.0 to 29.0 years. The male to female ratio was 82:59 and there were 116 whites, 20 African-Americans, and 5 Asian-Americans. The median follow-up was 2.0 years (range, 0.4 to 18.3 years). All patients had evidence by bone marrow aspiration, MIBG scans, or both of marrow infiltration. MYCN was determined in 102 patients (72%) and was amplified in 32 (31%) of these. All patients underwent imaging studies of cortical bone (bone survey

Discussion

The gold standard for testing the hypothesis that primary tumor removal affects outcome is a prospective randomized trial in which half the patients receive surgery and half do not. Currently, such a trial is not feasible because of the very poor prognosis in these patients (30% event-free survival) and the low probability that salvage therapy will work. Thus, the pediatric surgical oncologist is confronted with removing these often difficult tumors without strong evidence of efficacy from

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