Abstract
BackgroundAlthough several studies have been conducted on the role of surgery in localized neuroblastoma, the impact of surgical timing and extent of primary tumor resection on outcome in high-risk patients remains controversial.MethodsPatients from the German neuroblastoma trial NB97 with localized neuroblastoma INSS stage 1–3 age > 18 months were included for retrospective analysis. Imaging reports were reviewed by two independent physicians for Image Defined Risk Factors (IDRF). Operation notes and corresponding imaging reports were analyzed for surgical radicality. The extent of tumor resection was classified as complete resection (95–100%), gross total resection (90–95%), incomplete resection (50–90%), and biopsy (<50%) and correlated with local control rate and outcome. Patients were stratified according to the International Neuroblastoma Risk Group (INRG) staging system. Survival curves were estimated according to the method of Kaplan and Meier and compared by the log-rank test.ResultsA total of 179 patients were included in this study. 77 patients underwent more than one primary tumor operation. After best surgery, 68.7% of patients achieved complete resection of the primary tumor, 16.8% gross total resection, 14.0% incomplete surgery, and 0.5% biopsy only. The cumulative complication rate was 20.3% and the surgery associated mortality rate was 1.1%. Image defined risk factors (IDRF) predicted the extent of resection. Patients with complete resection had a better local-progression-free survival (LPFS), event-free survival (EFS) and OS (overall survival) than the other groups. Subgroup analyses showed better EFS, LPFS and OS for patients with complete resection in INRG high-risk patients. Multivariable analyses revealed resection (complete vs. other), and MYCN (non-amplified vs. amplified) as independent prognostic factors for EFS, LPFS and OS.ConclusionsIn patients with localized neuroblastoma age 18 months or older, especially in INRG high-risk patients harboring MYCN amplification, extended surgery of the primary tumor site improved local control rate and survival with an acceptable risk of complications.
Highlights
Several studies have been conducted on the role of surgery in localized neuroblastoma, the impact of surgical timing and extent of primary tumor resection on outcome in high-risk patients remains controversial
All patients registered by the German prospective clinical trial NB97 between November 1, 1996, and September 30, 2004, were included in this analysis when they met the following criteria: stage 1–3 neuroblastoma diagnosed according to the International Neuroblastoma Staging System criteria [7]; age at diagnosis >18 months but less than 21 years
Local progression–free survival (LPFS) was calculated from diagnosis to relapse or progression of the Patients Among 1121 patients in the NB97 trial, 191 patients with International Neuroblastoma Staging System (INSS) stage 1–3 >18 months were eligible for analysis
Summary
Several studies have been conducted on the role of surgery in localized neuroblastoma, the impact of surgical timing and extent of primary tumor resection on outcome in high-risk patients remains controversial. Despite the advances in multidrug therapy, surgery still plays a major role in treatment of neuroblastoma (NB) [2]. Children presenting with localized disease have an overall better prognosis, mainly depending on the degree of tumor resection [3]. Biological and clinical prognostic markers may help to stratify risks and guide therapy in these patients, but prospective randomized trials with surgical endpoints are still missing. We analyzed the impact of the extent of tumor resection on outcome of patients older than 18 months with localized nonmetastatic NB aged, who were treated according to the German prospective clinical trial NB97
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