Abstract
We report male monocygotic twins with concordant desmoplastic medulloblastoma diagnosed at the age of 20 months. Both tumours were completely removed. As chromosomal loci 17p13 and 9831 are frequently altered in medulloblastoma these regions were analysed in both tumours in detail using restriction fragment length polymorphism and microsatellite analysis. No common aberration was found. The c-myc gene on chromosome 8g21 was not amplified.
Similar content being viewed by others
Abbreviations
- APC :
-
adenomatous polyposis coli
- LOH :
-
loss of heterozygosity
- NBCCS :
-
nevoid basal cell carcinoma syndrome
- PBL :
-
peripheral blood lymphocyte
- PCR :
-
polymerase chain reaction
- PNET :
-
primitive neuro-ectodermal tumour
References
Chenevix-Trench G, Wicking C, Berkman J, Sharpe H, Hockey A, Haan E, Oley C, Ravine D, Tume A, Goldgar D, Searly J, Wainwright B (1993) Further localization of the gene for nevoid basal cell carcinoma syndrome (NBCCS) in 15 australasian families: linkage and loss of heterozygosity. Am J Hum Genet 53:760–767
Cogen P, Daneshvar L, Metzger A, Duyk G, Edwards M, Sheffield V (1992) Involvement of multiple chromosome 17p loci in medulloblastoma tumorigenesis. Am J Hum Genet 50:584–589
Du Manoir S, Speicher M, Joos S, Schröck E, Popp S, Döhner H, Kovacs G, Robert-Nicoud M, Lichter P, Cremer T (1993) Detection of complete and partial chromosome gains and losses by comparative genomic in situ hybridization. Hum Genet 90:590–610
Evans D, Farndon P, Burnell L, Gattamaneni H, Birch J (1991) The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma. Br J Cancer 64:959–961
Farwell J, Flannery B (1984) Cancer in relatives of children with central-nervous-system neoplasms. N Engl J Med 311:749–753
Fujii Y, Hongo T, Hayashi Y (1994) Chromosome analysis of brain tumors in childhood. Genes Chrom Cancer 11:205–215
Gailani M, Bale S, Leffell D, DiGiovanna J, Peck G, Poliak S, Drum A, Pastakia B, McBride O, Kase R, Greene M, Mulvihill J, Bale A (1992) Developmental defects in Gorlin syndrome related to a putative tumor suppressor gene on chromosome 9. Cell 69:111–117
Gerken S, Albertsen H, Elsner T, Ballard L, Holik P, Lawrence E, Moore M, Zhao X, White R (1995) A strategy for constructing high-resolution genetic maps of the human genome: a genetic map of chromosome 17p, ordered with meiotic breakpoint-mapping panels. Am J Hum Gene 56:484–499
Gill-Super H, Rothberg P, Kobayashi H, Freeman A, Diaz M, Rowle J (1994) Clonal, nonconstitutional rearrangements of the MLL gene in infant twins with acute lymphoblastic leukemia: in utero chromosome rearrangement of 11g23. Blood 83:641–644
Goldstein A, Stewart C, Bale A, Bale S, Dean M (1994) Localization of the gene for the nevoid basal cell carcinoma syndrome. Am J Hum Genet 53:760–767
Gorlin R, Goltz R (1962) Multiple nevoid basal-cell epitheiioma, jaw cysts and bifid rib. A syndrome. N Engl J Med 262:908–912
Gorlin R, Vickers R, Kelln E, Williamso J (1965) The multiple basalcell nevi syndrome: An analysis of a syndrome consisting of multiple nevoid basal-cell carcinoma, jaw cysts, skeletal anomalies, medulloblastoma, and hyporesponsiveness to parathormone. Cancer 18:89–104
Gyapay G, Morissette J, Vignal A, Dib C, Fizames C, Millasseau P, Marc S, Bernardi G, Lathrop M, Weissenbach J (1994) The 1993-94 Généthon human genetic linkage map. Nature Genet 7:246–339
Hamilton S, Liu B, Parsons R, Papadopoulos N, Jen J, 15 others (1995) The molecular basis of Turcot's syndrome. N Engl J Med 332:839–847
Hung K-L, Wu C-M, Huang J-S, How S-W (1990) Familial medulloblastoma in siblings: report in one family and review of the literature. Surg Neurol 33:341–346
Jeffreys A, Wilson V, Thein S (1985) Individual-specific “fingerprints” of human DNA. Nature 314:76–79
Kallioniemi A, Kallioniemi O-P, Sudar D, Rutovitz D, Gray J, Waldman F, Pinkel D (1992) Comparative genomic hybridization for molecular cytogenetic analysis of solid tumors. Science 258:818–821
Karnes P, Tran T, Cui M, Raffel C, Gilles F, Barranger J, King K (1992) Cytogenetic analysis of 39 pediatric central nervous system tumors. Cancer Genet Cytogenet 59:12–19
Kwok S, Higuchi R (1989) Avoiding false positives with PCR. Nature 339:237–239
Lyall J, Furlong R, Yuille M, Goudie D, Leversha M, Affara N, Ferguson-Smith M (1992) A dinucleotide repeat polymorphism at the D9S127 locus. Nucleic Acids Res 20:925
McDonald J, Daneshvar L, Willert J, Matsumura K, Waldman F, Cogen P (1994) Physical mapping of chromosome 17p13.3 in the region of a putative tumor suppressor gene important in medulloblastoma. Genomics 23:229–232
MacGregor D, Ziff E (1990) Elevated c-myc expression in childhood medulloblastomas. Pediatr Res 28:63–68
Miller S, Dykes D, Polesky H (1988) A simple salting out procedure for extracting DNA from human nucleated cells. Nucleic Acids Res 16:1215
Mori T, Nagase H, Horii A, Miyoshi Y, Shimano T, Nakatsuru S, Aoki T, Arakawa H, Yanagisawa A, Ushio Y, et al. (1994) Germ-line and somatic mutations of the APC gene in patients with Turcot syndrome and analysis of APC mutations in brain tumors. Genes Chrom Cancer 9:168–172
Neumann E, Kalousek D, Norman N, Steinbok P, Cochrane D, Goddard K (1993) Cytogenetic analysis of 109 pediatric central nervous system tumors. Cancer Genet Cytogenet 71:40–49
Scheurlen W, von Stockhausen H-B, Kreth H (1991) Diagnose der Monozygotie bei Mehrlingen durch DNA-Fingerprinting. Monatsschr Kinderheilkd 139, 30–32
Scheurlen W, Krauss J, Kühl J (1995) No preferential loss of one parental allele of chromosome 17p13.3 in childhood medulloblastoma. Int J Cancer 63:372–374
Schofield D, West D, Anthony D, Marshal R, Sklar J (1995) Correlation of loss of heterozygosity at chromosome 9q with histological subtype in medulloblastomas. Am J Pathol 146:472–480
Schütz B, Scheurlen W, Krauss J, Manoir S du, Joos S, Bentz M, Lichter P (1995) Mapping of chromosomal gains and losses in primitive neuroectodermal tumors by comparative genomic hybridization. Genes Chrom Cancer (in press)
Tijssen C, Halprin M, Endtz L (1982) Familial brain tumors. A commented register. Chapter II, pp 10–73. Martinus Nijhoff Publishers, The Hague (1982)
Turcot J, Després J, St. Pierre M (1959) Malignant tumors of the central nervous system associated with familial polyposis of the colon: report of two cases. Dis Colon Rectum 2:465–468
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Scheurlen, W., Sörensen, N., Roggendorf, W. et al. Molecular analysis of medulloblastomas occurring simultaneously in monozygotic twins. Eur J Pediatr 155, 880–884 (1996). https://doi.org/10.1007/BF02282838
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02282838