Skip to main content
Log in

Molecular analysis of medulloblastomas occurring simultaneously in monozygotic twins

  • Hematology/Oncology
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

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

  1. 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

    Google Scholar 

  2. 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

    Google Scholar 

  3. 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

    Google Scholar 

  4. 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

    Google Scholar 

  5. Farwell J, Flannery B (1984) Cancer in relatives of children with central-nervous-system neoplasms. N Engl J Med 311:749–753

    Google Scholar 

  6. Fujii Y, Hongo T, Hayashi Y (1994) Chromosome analysis of brain tumors in childhood. Genes Chrom Cancer 11:205–215

    Google Scholar 

  7. 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

    Google Scholar 

  8. 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

    Google Scholar 

  9. 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

    Google Scholar 

  10. 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

    Google Scholar 

  11. Gorlin R, Goltz R (1962) Multiple nevoid basal-cell epitheiioma, jaw cysts and bifid rib. A syndrome. N Engl J Med 262:908–912

    Google Scholar 

  12. 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

    Google Scholar 

  13. 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

    Google Scholar 

  14. 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

    Google Scholar 

  15. 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

    Google Scholar 

  16. Jeffreys A, Wilson V, Thein S (1985) Individual-specific “fingerprints” of human DNA. Nature 314:76–79

    Google Scholar 

  17. 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

    Google Scholar 

  18. 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

    Google Scholar 

  19. Kwok S, Higuchi R (1989) Avoiding false positives with PCR. Nature 339:237–239

    Google Scholar 

  20. 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

    Google Scholar 

  21. 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

    Google Scholar 

  22. MacGregor D, Ziff E (1990) Elevated c-myc expression in childhood medulloblastomas. Pediatr Res 28:63–68

    Google Scholar 

  23. Miller S, Dykes D, Polesky H (1988) A simple salting out procedure for extracting DNA from human nucleated cells. Nucleic Acids Res 16:1215

    Google Scholar 

  24. 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

    Google Scholar 

  25. 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

    Google Scholar 

  26. Scheurlen W, von Stockhausen H-B, Kreth H (1991) Diagnose der Monozygotie bei Mehrlingen durch DNA-Fingerprinting. Monatsschr Kinderheilkd 139, 30–32

    Google Scholar 

  27. 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

    Google Scholar 

  28. 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

    Google Scholar 

  29. 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)

  30. Tijssen C, Halprin M, Endtz L (1982) Familial brain tumors. A commented register. Chapter II, pp 10–73. Martinus Nijhoff Publishers, The Hague (1982)

    Google Scholar 

  31. 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

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints 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

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02282838

Key words

Navigation