Cancer Report

Cancer : Mycosis fungoides/Sezary's syndrome
Summary
Cancer Name Mycosis fungoides/Sezary's syndrome
 
Clinics and Pathology
Epidemiology This is the most common form of cutaneous T-cell lymphoma. The annual incidence is around 0,3 cases per 100.000 in western countries. The median age at diagnosis is between 55 and 60 years, with a 2/1 male-to-female ratio.
Pathology The tumor cell is a small lymphocyte with cerebriform nucleus, clumped chromatin and inconspicuous nucleoli. Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabsecsses is the hallmark of the disease.
Treatment Phototherapy, radiation therapy and alpha interferon are the mainstay of treatment of cutaneous disease. Chemotherapy using various regimens was employed in cases displaying disseminated disease and in Sezary's syndrome with limited success.
Prognosis The clinical stage is the most important indicator. Patient with limited cutaneous disease have an excellent prognosis. Patient with cutaneous tumours, generalized erythroderma, with Sezary's syndrome or extracutaneous disease usually have a short survival, ranging from 1 to 4 years.
 
Related Genes
Gene Symbol RB1
Description From COSMIC:
 
Gene Symbol PTENP1
Description From COSMIC:
 
Gene Symbol N-RAS
Description From COSMIC:
 
Gene Symbol CDKN2A
Description From COSMIC:
 
Cytogenetics
Cytogenetics Molecular
  • The absence of breaks involving the TCR regions was confirmed by fluorescence in situ hybridization.
  • Comparative genomic hybridization studies showed chromosome imbalances in 56% of the cases. DNA losses occurred at 1p (38%), 17p (21%), 10q (15%), and 19 (15%). DNA gains involved 4q (18%), 18 (15%) and 17q (12%). 1p33-36 and 10q26 may represent regions of minimal recurrent deletion. On chromosome 1p, two regions of minimal common deletion at 1p36 (D1S228 marker) and 1p22 (D1S2766 marker) were defined by allelotyping.
  • The microarray technology allowed for the identification of gains of distinct oncogene copy numbers in the majority of cases: RAF1 at 3p25; CTSB at 8p22; PAK1 at 11q13; and JUNB at 19p13. Amplification of the latter oncogene was found in some cases with strong nuclear expression of the corresponding protein product.
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    Bibliography
    Title Amplification and overexpression of JUNB is associated with primary cutaneous T-cell lymphomas.
    Authors Mao X, Orchard G, Lillington DM, Russell-Jones R, Young BD, Whittaker SJ.
    Citations Blood 2003; 101: 1513-1519.
    PubMedID 10577857
     
    Title Recurring structural chromosome abnormalities in peripheral blood lymphocytes of patients with mycosis fungoides/Sezary syndrome.
    Authors Thangavelu M, Finn WG, Yelavarthi KK, Roenigk HH Jr, Samuelson E, Peterson L, Kuzel TM, Rosen ST.
    Citations Blood 1997; 89: 3371-3377.
    PubMedID 12207585
     
    Title Molecular cytogenetic detection of chromosomal breakpoints in T-cell receptor gene loci.
    Authors Gesk S, Martin-Subero JI, Harder L, Luhmann B, Schlegelberger B, Calasanz MJ, Grote W, Siebert R.
    Citations Leukemia 2003; 17: 738-745.
    PubMedID 12557225
     
    Title Molecular cytogenetic characterization of Sezary syndrome.
    Authors Mao X, Lillington DM, Czepulkowski B, Russell-Jones R, Young BD, Whittaker S.
    Citations Genes Chromosomes Cancer 2003; 36: 250-260.
    PubMedID 12393503
     
    Title Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sezary syndrome and mycosis fungoides.
    Authors Mao X, Lillington D, Scarisbrick JJ, Mitchell T, Czepulkowski B, Russell-Jones R, Young B, Whittaker SJ.
    Citations Br J Dermatol 2002; 147: 464-475.
    PubMedID 12682631
     
    Title Cutaneous T-cell lymphomas
    Authors Kim Y, Hoppe RT.
    Citations In: Magrath I (Ed) The non Hodgkin's lymphomas 2nd edition. Pp 907-926; Arnold, London 1997.
    PubMedID 9129044
     
    Source and Citation
    Source Atlas of Genetics and Cytogenetics in Oncology and Haematology
    Citation Cuneo A, Castoldi GL . Mycosis fungoides/Sezary's syndrome. Atlas Genet Cytogenet Oncol Haematol. May 2005 .
    URL : http://AtlasGeneticsOncology.org/Anomalies/MycosFungID2039.html
    URL http://AtlasGeneticsOncology.org/Anomalies/MycosFungID2039.html