KEGG   DISEASE: Mycosis fungoidesHelp
Entry
H01463                      Disease                                

Name
Mycosis fungoides
Description
Mycosis fungoides (MF) is the most common type of primary cutaneous T-cell lymphomas (CTCL), which are a heterogeneous group of malignancies derived from skin-homing T cells. MF presents in the skin with erythematous patches, plaques, and less frequently, tumours. Although the aetiologies of MF are unknown, important insights have been gained in the immunological and genetic perturbations that are associated with these diseases. Mutations in the p53, p15, p16, JunB, and PTEN genes generally occur in later-stage disease. Loss of normal apoptotic T-cell pathways has also been reported. Apoptosis is partly mediated by death receptors, notably Fas, which is part of the tumor necrosis factor family of receptors. Decreased and/or defective Fas expression by neoplastic T cells has been associated with advanced/aggressive disease and impaired Fas-mediated apoptosis.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Skin cancers
   H01463  Mycosis fungoides
  Cancers of haematopoietic and lymphoid tissues
   H01463  Mycosis fungoides
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature T-cell or NK-cell neoplasms
    Mature T-cell or NK-cell lymphoma, primary cutaneous specified types
     2B01  Mycosis fungoides
      H01463  Mycosis fungoides
BRITE hierarchy
Gene
p16/INK4a, ARF (mutation,deletion) [HSA:1029] [KO:K06621]
p15/INK4b (mutation, deletion) [HSA:1030] [KO:K04685]
PTEN (mutation) [HSA:5728] [KO:K01110]
p53 (mutation) [HSA:7157] [KO:K04451]
JUNB (mutation) [HSA:3726] [KO:K09028]
Fas (loss of expression) [HSA:355] [KO:K04390]
Nav3 (deletion) [HSA:89795]
c-MYC (amplification) [HSA:4609] [KO:K04377]
Drug
Triamcinolone acetonide [DR:D00983]
Dexamethasone [DR:D00292]
Dexamethasone sodium phosphate [DR:D00975]
Hydrocortisone [DR:D00088]
Hydrocortisone sodium succinate [DR:D00978]
Prednisolone [DR:D00472]
Prednisolone sodium phosphate [DR:D00981]
Prednisone [DR:D00473]
Methylprednisolone [DR:D00407]
Methylprednisolone sodium succinate [DR:D00751]
Methylprednisolone acetate [DR:D00979]
Cortisone acetate [DR:D00973]
Cyclophosphamide [DR:D00287]
Mechlorethamine hydrochloride [DR:D04872]
Carmustine [DR:D00254]
Methotrexate sodium [DR:D02115]
Vinblastine sulfate [DR:D01068]
Doxorubicin hydrochloride [DR:D01275]
Mogamulizumab [DR:D09761] (CCR4-directed)
Bexarotene [DR:D03106]
Denileukin diftitox [DR:D03682] (CD25 express)
Vorinostat [DR:D06320]
Comment
ICD-O-3.1: 9700/3 Mycosis fungoides
Other DBs
ICD-11: 2B01
ICD-10: C84
MeSH: D009182
Reference
  Authors
Hwang ST, Janik JE, Jaffe ES, Wilson WH
  Title
Mycosis fungoides and Sezary syndrome.
  Journal
Lancet 371:945-57 (2008)
DOI:10.1016/S0140-6736(08)60420-1
Reference
  Authors
Beyer M, Mobs M, Humme D, Sterry W
  Title
Pathogenesis of Mycosis fungoides.
  Journal
J Dtsch Dermatol Ges 9:594-8 (2011)
DOI:10.1111/j.1610-0387.2011.07635.x
Reference
  Authors
Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C
  Title
Primary cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers.
  Journal
J Am Acad Dermatol 70:205.e1-16; quiz 221-2 (2014)
DOI:10.1016/j.jaad.2013.07.049
Reference
  Authors
Laharanne E, Chevret E, Idrissi Y, Gentil C, Longy M, Ferrer J, Dubus P, Jouary T, Vergier B, Beylot-Barry M, Merlio JP
  Title
CDKN2A-CDKN2B deletion defines an aggressive subset of cutaneous T-cell lymphoma.
  Journal
Mod Pathol 23:547-58 (2010)
DOI:10.1038/modpathol.2009.196
Reference
  Authors
Smoller BR
  Title
Mycosis fungoides: what do/do not we know?
  Journal
J Cutan Pathol 35 Suppl 2:35-9 (2008)
DOI:10.1111/j.1600-0560.2008.01120.x
Reference
  Authors
Karenko L, Hahtola S, Paivinen S, Karhu R, Syrja S, Kahkonen M, Nedoszytko B, Kytola S, Zhou Y, Blazevic V, Pesonen M, Nevala H, Nupponen N, Sihto H, Krebs I, Poustka A, Roszkiewicz J, Saksela K, Peterson P, Visakorpi T, Ranki A
  Title
Primary cutaneous T-cell lymphomas show a deletion or translocation affecting NAV3, the human UNC-53 homologue.
  Journal
Cancer Res 65:8101-10 (2005)
DOI:10.1158/0008-5472.CAN-04-0366
Reference
(drug)
  Authors
Papadakis MA and McPhee SJ |(ed)
  Title
Current Medical Diagnosis & Treatment 2015, Fifty-Fourth Edition
  Journal
McGraw Hill Education
Reference
PMID:24841586 (drug)
  Authors
Wu PA, Huang V, Bigby ME
  Title
Interventions for mycosis fungoides: critical commentary on a Cochrane Systematic Review.
  Journal
Br J Dermatol 170:1015-20 (2014)
DOI:10.1111/bjd.12954
Reference
PMID:20568590 (drug)
  Authors
Galper SL, Smith BD, Wilson LD
  Title
Diagnosis and management of mycosis fungoides.
  Journal
Oncology (Williston Park) 24:491-501 (2010)
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