Biomaterial Database

Neuroblastomas of infancy exhibit a characteristic ganglioside pattern. 2001

K Kaucic, and N Etue, and B LaFleur, and W Woods, and S Ladisch

Glycobiology Program, Center for Cancer and Transplantation Biology, Children's National Medical Center, 111 Michigan Avenue NW, Washington, DC 20010, USA. kkaucic@cnmc.org

BACKGROUND Gangliosides are membrane-bound glycolipid molecules particularly prominent in neural tissue. Changes in ganglioside expression during embryologic development result from a shift in biosynthesis from the fetal b pathway to the adult a pathway. Tumor gangliosides may play a role in the clinical behavior of certain subtypes of neuroblastoma. Because neuroblastoma, which presents in infancy, has a different biologic and clinical phenotype than that which presents in older children, the authors determined whether differences in ganglioside biosynthesis exist between these two neuroblastoma subgroups. METHODS Sixty-eight tumor specimens (25 diagnosed by screening and 43 diagnosed clinically) were obtained from the Quebec Neuroblastoma Screening Project. Gangliosides were isolated and purified by solvent partitioning, separated by high performance thin-layer chromatography, and quantitated by scanning densitometry. The sum of a and b pathway gangliosides were determined for each tumor. RESULTS Gangliosides of the b (fetal) pathway predominated in both screened and clinically diagnosed tumors of patients younger than 1 year of age. Twenty-three of 25 screened patients (92%) and 21 of 23 patients with clinically diagnosed tumors at younger than 1 year of age (91%) had tumor b pathway ganglioside content greater than 60%. In contrast, tumors of only 8 of 20 patients 1 year or older (40%) had b pathway ganglioside predominance. Predominance of b pathway tumor gangliosides correlated with improved outcome. Event free survival was significantly higher among patients with b pathway ganglioside tumor content greater than 60% versus those with b pathway ganglioside tumor content less than 60% (118.1 +/- 3.9 months vs. 69.2 +/- 8.6 months, P < 0.01). CONCLUSIONS Fetal patterns of ganglioside biosynthesis predominate in neuroblastoma tumors from patients younger than 1 year of age and adult patterns of ganglioside biosynthesis predominate in tumors from older children, supporting the view that neuroblastoma consists of distinct but overlapping disorders, and that gangliosides may play a biologic role in the clinical differences among these patients.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D009447	Neuroblastoma	A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51)	Neuroblastomas
D002855	Chromatography, Thin Layer	Chromatography on thin layers of adsorbents rather than in columns. The adsorbent can be alumina, silica gel, silicates, charcoals, or cellulose. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)	Chromatography, Thin-Layer,Thin Layer Chromatography,Chromatographies, Thin Layer,Chromatographies, Thin-Layer,Thin Layer Chromatographies,Thin-Layer Chromatographies,Thin-Layer Chromatography
D005732	Gangliosides	A subclass of ACIDIC GLYCOSPHINGOLIPIDS. They contain one or more sialic acid (N-ACETYLNEURAMINIC ACID) residues. Using the Svennerholm system of abbrevations, gangliosides are designated G for ganglioside, plus subscript M, D, or T for mono-, di-, or trisialo, respectively, the subscript letter being followed by a subscript arabic numeral to indicated sequence of migration in thin-layer chromatograms. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1997)	Ganglioside,Sialoglycosphingolipids
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D016019	Survival Analysis	A class of statistical procedures for estimating the survival function (function of time, starting with a population 100% well at a given time and providing the percentage of the population still well at later times). The survival analysis is then used for making inferences about the effects of treatments, prognostic factors, exposures, and other covariates on the function.	Analysis, Survival,Analyses, Survival,Survival Analyses
D018709	Statistics, Nonparametric	A class of statistical methods applicable to a large set of probability distributions used to test for correlation, location, independence, etc. In most nonparametric statistical tests, the original scores or observations are replaced by another variable containing less information. An important class of nonparametric tests employs the ordinal properties of the data. Another class of tests uses information about whether an observation is above or below some fixed value such as the median, and a third class is based on the frequency of the occurrence of runs in the data. (From McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1284; Corsini, Concise Encyclopedia of Psychology, 1987, p764-5)	Kolmogorov-Smirnov Test,Kruskal-Wallis H Statistic,Mann-Whitney U Test,Rank-Sum Tests,Spearman Rank Correlation Coefficient,Wilcox Test,Wilcoxon Rank Test,Non-Parametric Statistics,Nonparametric Statistics,Statistics, Non-Parametric,Kolmogorov Smirnov Test,Mann Whitney U Test,Non Parametric Statistics,Rank Sum Tests,Rank Test, Wilcoxon,Rank-Sum Test,Statistics, Non Parametric,Test, Kolmogorov-Smirnov,Test, Mann-Whitney U,Test, Rank-Sum,Test, Wilcox,Test, Wilcoxon Rank,Tests, Rank-Sum,U Test, Mann-Whitney