Neuroblastoma originating from extra-abdominal sites: association with favorable clinical and biological features

Abstract

Neuroblastomas originating from different sites might have different clinical and biological characteristics. In the present study, the clinical (age, sex and stage) and biological (N-myc amplification, Shimada pathology and levels of lactate dehydrogenase, ferritin and neuron-specific enolase) characteristics of patients with newly diagnosed neuroblastoma were compared according to the site of tumor origin (extra-abdominal versus abdominal). The event-free survival rate (EFS) was also compared between the two groups. Among 143 neuroblastomas, 115 tumors originated from the abdomen, 26 from extra-abdominal sites and 2 from unknown primary sites. Frequencies of stage 4 tumor and N-myc amplified tumor were lower in the extra-abdominal group than in the abdominal group (34.6% vs. 60.0%, P=0.019 and 4.2% vs. 45.0%, P<0.001, respectively). Levels of lactate dehydrogenase, ferritin and neuron-specific enolase were significantly lower in the extra-abdominal group than in the abdominal group. The probability of 5-yr EFS (+/-95% confidence interval) was higher in the extra-abdominal group than in the abdominal group (94.4+/-10.6% vs. 69.4+/-9.4%, P=0.026). Taken together, neuroblastomas originating from extra-abdominal sites might be associated with more favorable clinical and biological characteristics and a better outcome than neuroblastomas originating from abdomen.

Keywords: Neuroblastoma; Prognosis; Thorax; Transplantation, Autologous.

Fig. 1

Levels of serum LDH (A), ferritin (B), NSE (C) and 24-hr urine VMA (D) were lower in the extra-abdominal group than in the abdominal group.

Fig. 2

EFS rate with respect to tumor site of origin. A. The probability of 5-yr EFS was higher in patients with extra-abdominal tumors than in those with abdominal tumors (94.4±10.6% vs. 69.4±9.4%, P=0.026). B. When the analysis was confined to only the patients older than 1 yr of age at diagnosis, the probability of 5-yr EFS was also higher in patients with extra-abdominal tumor than in those with abdominal tumor (92.3±14.5% vs. 65.3±11.7%, P=0.047). C. Similarly, when the analysis was confined to only the patients with stage 4 tumor, the probability of 5-yr EFS was higher in patients with extra-abdominal tumors compared to those with abdominal tumors (87.5±22.9% vs. 59.2±12.8%, P=0.133), however, this difference was not significant.