title: 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.
authors: Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors: Ki Woong
authors: KW
authors: Sung
authors: Keon Hee
authors: KH
authors: Yoo
authors: Hong Hoe
authors: HH
authors: Koo
authors: Ju Youn
authors: JY
authors: Kim
authors: Eun Joo
authors: EJ
authors: Cho
authors: Yeon Lim
authors: YL
authors: Seo
authors: Jhingook
authors: J
authors: Kim
authors: Suk Koo
authors: SK
authors: Lee
mesh_terms: D018572:Disease-Free Survival; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008297:Male; D009447:Neuroblastoma / Q000175:diagnosis* / Q000628:therapy; D010751:Phosphopyruvate Hydratase / Q000097:blood; D011379:Prognosis; D016896:Treatment Outcome; D014642:Vanilmandelic Acid / Q000652:urine
chemical_list: D014642:Vanilmandelic Acid; D005293:Ferritins; D007770:L-Lactate Dehydrogenase; D010751:Phosphopyruvate Hydratase