J Korean Med Sci.  2009 Jun;24(3):461-467. 10.3346/jkms.2009.24.3.461.

Neuroblastoma Originating from Extra-abdominal Sites: Association with Favorable Clinical and Biological Features

• Sung KW
• Yoo KH
• Koo HH
• Kim JY
• Cho EJ
• Seo YL
• Kim J
• Lee SK

• KMID: 1779164
• DOI: http://doi.org/10.3346/jkms.2009.24.3.461

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.

Keyword

Neuroblastoma; Prognosis; Thorax; Transplantation, Autologous

MeSH Terms

Disease-Free Survival
Female
Ferritins/blood
Humans
L-Lactate Dehydrogenase/blood
Male
Neuroblastoma/*diagnosis/therapy
Phosphopyruvate Hydratase/blood
Prognosis
Treatment Outcome
Vanilmandelic Acid/urine

Figure

• 

  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.

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  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.

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