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Biological characteristics of neuroblastoma with partial deletion in the short arm of chromosome 1.
Med Pediatr Oncol. 2001 Jan; 36(1):67-74.MP

Abstract

BACKGROUND

Neuroblastoma shows remarkable heterogeneity, resulting in favorable and unfavorable outcomes. It is well known that almost all cases with MYCN amplification have a poor prognosis. We have previously reported that unfavorable tumors show high telomerase activity, whereas favorable tumors show low or nil activity. We also found that the unfavorable neuroblastoma often have a loss of heterozygosity (LOH) at the MYCL locus.

PROCEDURE

To clarify the biological and clinical profiles of tumors with genetic abnormalities of the short arm of chromosome 1, we performed deletion mapping on 1p on 92 neuroblastoma tissues and corresponding noncancerous samples obtained from 92 cases for 24 micro- or minisatellite loci.

RESULTS

LOH was detected in at least one locus of 1p in 43 (47%) cases. All samples were classified into four groups according to the deleted pattern: interstitial deletion (group I, n = 20), short terminal deletion (group ST, n = 6), large terminal deletion (group LT, n = 17), and without detectable deletion (group N, n = 49). All group I cases, whose SRO (shortest region of overlap) was at 1p36.1-2, survived disease free, and none of them showed MYCN amplification or high telomerase activity except for one case. On the other hand, in group LT cases, who showed a large terminal deletion from D1S162 (1p32-pter), including the SRO of group 1, only 5 out of 17 have survived disease free, and 13 showed MYCN amplification or high telomerase activity. The six group ST cases showed small terminal deletion from 1p36.3 with modest prognosis, similar to the group N.

CONCLUSIONS

Thus, we propose three loci, 1p36.1-2, 1p32-34, and 1p36.3, as the candidate loci of neuroblastoma suppressor genes on chromosome 1p responsible for groups I, LT, and ST, respectively. Among them, the 1p32-34 locus may be associated with aggressiveness of tumor progression, possibly due to MYCN amplification and/or telomerase reactivation, while the remaining two loci may not.

Authors+Show Affiliations

Department of General Medicine, Hiroshima University School of Medicine, Japan. eiso@mcai.med.hiroshima-u.ac.jpNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

11464909

Citation

Hiyama, E, et al. "Biological Characteristics of Neuroblastoma With Partial Deletion in the Short Arm of Chromosome 1." Medical and Pediatric Oncology, vol. 36, no. 1, 2001, pp. 67-74.
Hiyama E, Hiyama K, Ohtsu K, et al. Biological characteristics of neuroblastoma with partial deletion in the short arm of chromosome 1. Med Pediatr Oncol. 2001;36(1):67-74.
Hiyama, E., Hiyama, K., Ohtsu, K., Yamaoka, H., Fukuba, I., Matsuura, Y., & Yokoyama, T. (2001). Biological characteristics of neuroblastoma with partial deletion in the short arm of chromosome 1. Medical and Pediatric Oncology, 36(1), 67-74.
Hiyama E, et al. Biological Characteristics of Neuroblastoma With Partial Deletion in the Short Arm of Chromosome 1. Med Pediatr Oncol. 2001;36(1):67-74. PubMed PMID: 11464909.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Biological characteristics of neuroblastoma with partial deletion in the short arm of chromosome 1. AU - Hiyama,E, AU - Hiyama,K, AU - Ohtsu,K, AU - Yamaoka,H, AU - Fukuba,I, AU - Matsuura,Y, AU - Yokoyama,T, PY - 2001/7/24/pubmed PY - 2001/8/17/medline PY - 2001/7/24/entrez SP - 67 EP - 74 JF - Medical and pediatric oncology JO - Med Pediatr Oncol VL - 36 IS - 1 N2 - BACKGROUND: Neuroblastoma shows remarkable heterogeneity, resulting in favorable and unfavorable outcomes. It is well known that almost all cases with MYCN amplification have a poor prognosis. We have previously reported that unfavorable tumors show high telomerase activity, whereas favorable tumors show low or nil activity. We also found that the unfavorable neuroblastoma often have a loss of heterozygosity (LOH) at the MYCL locus. PROCEDURE: To clarify the biological and clinical profiles of tumors with genetic abnormalities of the short arm of chromosome 1, we performed deletion mapping on 1p on 92 neuroblastoma tissues and corresponding noncancerous samples obtained from 92 cases for 24 micro- or minisatellite loci. RESULTS: LOH was detected in at least one locus of 1p in 43 (47%) cases. All samples were classified into four groups according to the deleted pattern: interstitial deletion (group I, n = 20), short terminal deletion (group ST, n = 6), large terminal deletion (group LT, n = 17), and without detectable deletion (group N, n = 49). All group I cases, whose SRO (shortest region of overlap) was at 1p36.1-2, survived disease free, and none of them showed MYCN amplification or high telomerase activity except for one case. On the other hand, in group LT cases, who showed a large terminal deletion from D1S162 (1p32-pter), including the SRO of group 1, only 5 out of 17 have survived disease free, and 13 showed MYCN amplification or high telomerase activity. The six group ST cases showed small terminal deletion from 1p36.3 with modest prognosis, similar to the group N. CONCLUSIONS: Thus, we propose three loci, 1p36.1-2, 1p32-34, and 1p36.3, as the candidate loci of neuroblastoma suppressor genes on chromosome 1p responsible for groups I, LT, and ST, respectively. Among them, the 1p32-34 locus may be associated with aggressiveness of tumor progression, possibly due to MYCN amplification and/or telomerase reactivation, while the remaining two loci may not. SN - 0098-1532 UR - https://www.unboundmedicine.com/medline/citation/11464909/Biological_characteristics_of_neuroblastoma_with_partial_deletion_in_the_short_arm_of_chromosome_1_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0098-1532&date=2001&volume=36&issue=1&spage=67 DB - PRIME DP - Unbound Medicine ER -