Cancer Report

Cancer : Burkitt's lymphoma (BL)
Cancer Name Burkitt's lymphoma (BL)
Clinics and Pathology
Epidemiology Most common in children (1/3 of lymphomas). In adult it accounts for 3-4% of all lymphomas in western countries and it is frequently associated with immunodeficiency
  • The lymphoma consists of a monomorphic infiltrate of the lymph node by medium-sized cells showing round nuclei with several nucleoli and basophilic cytoplasm. Numerous benign macrophages confer a histologic pattern referred to as ?tarry sky? Involvement of the peripheral blood and bone marrow may occur.
  • The related form ?urkitt-like?lymphoma shows intermediate features between diffuse large cell lymphoma and BL and probably includes different disease entities. It was suggested by the WHO panel that only those cases with c-MYC rearrangement and/or a >99% proliferation fraction as demonstrated by Ki-67 positivity should be classified as Burkitt-like lymphoma
  • Treatment Aggressive regimens specifically designed for this lymphoma must be used
    Prognosis If treated promptly with appropriate regimens the majority of patients can be cured
    Related Genes
    Gene Symbol ARHH
    Description From OMIM: "Genomic instability promotes tumorigenesis through defective chromosome segregation and DNA mismatch repair inactivation. By screening 18 loci for mutations, Pasqualucci et al. (2001) identified changes in the germline sequences of PIM1 (164960), MYC (190080), ARHH, and/or PAX5 (167414), in addition to BCL6, in a majority of diffuse large-cell lymphomas (DLCLs; see 601889). No mutations in PIM1, MYC, ARHH, and PAX5 were detected in germinal-center lymphocytes, naive B cells, or B-cell malignancies other than DLCLs. ARHH mutations, which were observed in 46% of DLCLs, resided within noncoding sequences, suggesting an effect on regulation of ARHH expression. FISH analysis indicated that hypermutation in these genes is not due to chromosomal translocation, as seen in Burkitt lymphoma (113970). Chromosomal translocation, however, may be an outcome of hypermutation. Specific features of the hypermutation process, including the predominance of single nucleotide substitutions with occasional deletions or duplications, a preference for transitions over transversions, and a specific motif targeting RGYW, were recognizable in each of the hypermutated loci"
    Gene Symbol BCL7A
    Description From OMIM: "Chromosome 12q24.1 is the site of a recurrent breakpoint in high-grade B-cell non-Hodgkin lymphoma (BNHL). To identify the gene(s) involved in 12q24.1, molecular cloning of a 3-way translocation t(8;14;12)(q24.1;q32.3;q24.1) in a Burkitt lymphoma cell line was performed by Zani et al. (1996); all 4 translocation breakpoints were cloned and sequenced. Analysis of the der(12)(12;14)(q24.1;q32.3) breakpoint showed a CpG island from 12q24.1 juxtaposed in a tail-to-tail configuration with a productively rearranged V-D-J heavy chain gene. A total of 4.5 kb of genomic DNA was shown to contain a potential 92-bp exon within the centromeric boundary of the CpG island. This was used as a probe to identify an RNA transcript of 3.8 kb expressed at low levels in a wide variety of normal tissues. The longest open reading frame predicted a serine-rich protein of 231 amino acids. This protein, designated BCL7A by the authors, exhibits no recognizable protein motifs but shows homology with the actin-binding protein, caldesmon (114213). In the Burkitt lymphoma cell line Wein 133 containing the 3-way translocation, the BCL7A breakpoint occurred within the first intron and resulted in a free-of-fusion transcript between MYC (190080) and BCL7A, with exon 1 of BCL7A being replaced by MYC exon 1. The normal, untranslocated allele of BCL7A was also expressed without mutation. One of the 11 other BNCLs with 12q24.1 cytogenetic abnormalities showed biallelic rearrangement within the first intron of BCL7A, which was adjacent to the breakpoint observed in Wein 133. Zani et al. (1996) concluded that disruption of the amino-terminus of BCL7A defined a new mechanism in the pathogenesis of a subset of high-grade BNHL."
    Gene Symbol IGH
    Description From cancer gene census of CGP: "
  • The t(8,14) and the variant t(8;22) and t(2;8) juxtapose IgH sequences and the c-MYC oncogene, bringing about its constitutional expression. "
    Gene Symbol PTENP1
    Description From COSMIC:
    Gene Symbol RBL2
    Description "This oncosuppressor gene is involved in, Breast Cancer ,, Burkitt's lymphomas ,, Choroidal melanoma ,, Endometrial Cancer ,, Lung Cancer , Malignant Lymphomas, Mesothelioma,, Glioblastoma ,, Ovarian Cancer , Oral malignant melanoma, Vulvar Carcinoma."
    Gene Symbol MYC
    Description From cancer gene census of CGP: "
  • The t(8,14) and the variant t(8;22) and t(2;8) juxtapose IgH sequences and the c-MYC oncogene, bringing about its constitutional expression. "
    Gene Symbol N-RAS
    Description From COSMIC:
    Gene Symbol TOPORS
    Description From COSMIC:
    Gene Symbol CDKN2A
    Description From COSMIC:
    Gene Symbol BCL5
    Description From maunal review:
    Gene Symbol K-RAS
    Description From COSMIC:
    Gene Symbol IGL@
    Description From cancer gene census of CGP
    Gene Symbol P53
    Description " The 17p deletion may have a correlation with p53 loss of function, determined by deletion of one allele and inactivating mutation of the remaining allele. "
    Gene Symbol IGK
    Description From cancer gene census of CGP
    Gene Symbol RAP2A
    Description From OMIM: "By screening a Raji Burkitt lymphoma cell line cDNA library with Drosophila Dras3 cDNA, Pizon et al. (1988) cloned RAP1A (179520) and RAP2A. The deduced 183-amino acid protein has a calculated molecular mass of 20.7 kD. It contains domains required for GTP binding and a Ras (see 190020)-like C-terminal motif for posttranslational lipid binding and subsequent anchoring to the plasma membrane. Northern blot analysis of lymphocyte mRNA detected transcripts of 2.2, 2.5, and 4.6 kb. "
    Title Small noncleaved cell lymphomas (Burkitt's and Burkitt-like lymphomas).
    Authors Magrath I.
    Citations In: Magrath I (ed): The non Hodgkin's lymphomas. Pp781-811. Arnold, London 1997.
    PubMedID 10334544
    Title Small noncleaved, non Burkitt's (Burkitt-like) lymphoma: Cytogenetics predict outcome and reflect clinical presentation.
    Authors Macpherson N, Lesak D, Klasa R, Horsman D, Connors JM, Barnett M, Gascoyne RD.
    Citations J Clin Oncol 1999; 17: 1558-1567.
    PubMedID 10334544
    Title High frequency of EBV association with non-random abnormalities of the chromosome region 1q21-25 in AIDS-related Burkitt's lymphoma-derived cell lines.
    Authors Polito P, Cilia AM, Gloghini A, Cozzi M, Perin T, De Paoli P, Gaidano G, Carbone A.
    Citations Int J Cancer. 1995; 61: 370-4.
    PubMedID 9446660
    Title Molecular genetics of malignant lymphoma. In: Fo?R (ed): Reviews in clinical and experimental hematology.
    Authors Gaidano G.
    Citations Forum Service Editore, Genova and Martin Dunitz, London, 1997.
    PubMedID 10577857
    Title World Health Organization classification of neoplastic diseases of hematopoietic and lymphoid tissues: Report of the clinical advisory committee - Airlie House, Virginia, Novembre 1997.
    Authors Harris NNL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD.
    Citations J Clin Oncol 1999; 17: 3835-3849
    PubMedID 10334544
    Title A revised European-American classification of lymphoid neoplasms: a proposal from the international lymphoma study group.
    Authors Harris NL, Jaffe ES, Stein H, Banks PM, Chan JKC, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC, Grogan TM, Isaacson PG, Knowles DM, Mason DY, Muller-Hermelink HK, Pileri SA, Piris MA, Ralfkiaer E, Warnke RA.
    Citations Blood 1994; 84: 1361.
    PubMedID 8068936
    Title Chromosomal abnormalities in adult non-endemic Burkitt's lymphoma and leukemia: 22 new reports and a review of 148 cases from the literature.
    Authors Kornblau SM, Goodacre A, Cabanillas F.
    Citations Hematol Oncol. 1991; 9:63-78.
    PubMedID 1869243
    Title Clinicopthogenetic significance of chromosomal abnormalities in patients with blastic peripheral B-cell lymphoma.
    Authors Schlegelberger B, Zwingers T, Harder T, Nowotny H, Siebert R, Vesely M, Bartels H, Sonnen R, Hopfinger G, Nader A, Ott G, Muller-Hermelink K, Feller A, Heinz R, for the Kiel-Wien-Lymphoma Study Group.
    Citations Blood 1999; 94: 3114-3120.
    PubMedID 1869243
    Title Application of interphase fluorescence in situ Hybridization for the detection of the Burkitt translocation t(8;14)(q24;q32) in B-cell lymphomas.
    Authors Siebert R, Matthiesen P, Harder S, Zhang Y, Borowski A, Zuhlke-Jenisch R, Metzke S, Joos S, Weber-Matthiesen K, Grote W, Schlegelberger B.
    Citations Blood. 1998; 91:984-90.
    PubMedID 10556197
    Title Catalogue of chromosome aberrations in cancer (5th edition).
    Authors Mitelmam F (ed).
    Citations Wiley Liss, New York 1994.
    PubMedID 7729949
    Title Molecular cloning of complex chromosomal translocation t(8;14;12)(q24.1;q32.3;q24.1) in a Burkitt lymphoma cell line defines a new gene (BCL7A) with homology to caldesmon.
    Authors Zani, V. J.; Asou, N.; Jadayel, D.; Heward, J. M.; Shipley, J.; Nacheva, E.; Takasuki, K.; Catovsky, D.; Dyer, M. J. S.
    Citations Blood 87: 3124-3134, 1996.
    PubMedID 8605326
    Title Hypermutation of multiple proto-oncogenes in B-cell diffuse large-cell lymphomas.
    Authors "Pasqualucci, L.; Neumeister, P.; Goossens, T.; Nanjangud, G.; Chaganti, R. S. K.; Kuppers, R.; Dalla-Favera, R. "
    Citations "Nature 412: 341-346, 2001. "
    PubMedID 11460166
    Title Human cDNAs rap1 and rap2 homologous to the Drosophila gene Dras3 encode proteins closely related to ras in the 'effector' region.
    Authors "Pizon, V.; Chardin, P.; Lerosey, I.; Olofsson, B.; Tavitian, A."
    Citations "Oncogene 3: 201-204, 1988. "
    PubMedID 3045729
    Source and Citation
    Source Atlas of Genetics and Cytogenetics in Oncology and Haematology
    Citation Cuneo A, Castoldi GL . Burkitt's lymphoma (BL). Atlas Genet Cytogenet Oncol Haematol. March 2001 .
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