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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 - J. Korean Med. Sci. (2009)

Bottom Line: The event-free survival rate (EFS) was also compared between the two groups.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).

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

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.

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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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Figure 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.

Mentions: Tumors relapsed or progressed in 16 patients and treatment-related mortality occurred in 14 patients. Therefore, 111 out of 141 patients remained event free with a median follow-up of 47 months (range 1-131) from diagnosis. The probability of 5-yr OS and EFS rate (±95% CI) for all 141 patients was 76.3±8.5% and 73.9±8.1%, respectively. The probability of 5-yr EFS rates and the results of the unstratified log-rank test with respect to the clinical and biological characteristics are listed in Table 2. The probability of 5-yr EFS rate in patients with extra-abdominal tumors was higher than in patients with abdominal tumors (94.4±10.6% vs. 69.4±9.4%, P=0.026). Fig. 2A shows the EFS curves for patients with extra-abdominal and abdominal tumors. All but one patient in the extra-abdominal group are currently event free. When the analysis was confined to only the patients older than 1 yr of age at diagnosis, the probability of 5-yr EFS rate was higher in patients with extra-abdominal tumors than in those with abdominal tumors (92.3±14.5% vs. 65.3±11.7%, P=0.047, Fig. 2B). Similarly, when the analysis was confined to only the patients with stage 4 tumors, the probability of 5-yr EFS rate was higher in patients with extra-abdominal tumors than in those with abdominal tumors (87.5±22.9% vs. 59.2±12.8%, P=0.133, Fig. 2C), albeit this difference was not significant. All five patients with both extra-thoracic and extra-abdominal tumors remained event free with a median follow-up of 123 months (range 10-131) from diagnosis.


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 - J. Korean Med. Sci. (2009)

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.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2698193&req=5

Figure 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.
Mentions: Tumors relapsed or progressed in 16 patients and treatment-related mortality occurred in 14 patients. Therefore, 111 out of 141 patients remained event free with a median follow-up of 47 months (range 1-131) from diagnosis. The probability of 5-yr OS and EFS rate (±95% CI) for all 141 patients was 76.3±8.5% and 73.9±8.1%, respectively. The probability of 5-yr EFS rates and the results of the unstratified log-rank test with respect to the clinical and biological characteristics are listed in Table 2. The probability of 5-yr EFS rate in patients with extra-abdominal tumors was higher than in patients with abdominal tumors (94.4±10.6% vs. 69.4±9.4%, P=0.026). Fig. 2A shows the EFS curves for patients with extra-abdominal and abdominal tumors. All but one patient in the extra-abdominal group are currently event free. When the analysis was confined to only the patients older than 1 yr of age at diagnosis, the probability of 5-yr EFS rate was higher in patients with extra-abdominal tumors than in those with abdominal tumors (92.3±14.5% vs. 65.3±11.7%, P=0.047, Fig. 2B). Similarly, when the analysis was confined to only the patients with stage 4 tumors, the probability of 5-yr EFS rate was higher in patients with extra-abdominal tumors than in those with abdominal tumors (87.5±22.9% vs. 59.2±12.8%, P=0.133, Fig. 2C), albeit this difference was not significant. All five patients with both extra-thoracic and extra-abdominal tumors remained event free with a median follow-up of 123 months (range 10-131) from diagnosis.

Bottom Line: The event-free survival rate (EFS) was also compared between the two groups.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).

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

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.

Show MeSH
Related in: MedlinePlus