| H01463 | |
| H number | H01463 |
| 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 |
| Network | - |
| 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] [KO:K23919] c-MYC (amplification) [HSA:4609] [KO:K04377] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| 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] Methotrexate sodium [DR:D02115] Vinblastine sulfate [DR:D01068] Brentuximab vedotin [DR:D09587] (CD30 expressing) Mogamulizumab [DR:D09761] (CCR4-directed) Bexarotene [DR:D03106] Vorinostat [DR:D06320] Romidepsin [DR:D06637] Denileukin diftitox [DR:D03682] (CD25 express) |
| Comment | - |
| Other DBs | ICD-11: 2B01 ICD-10: C84 MeSH: D009182 |
| Reference | PMID:18342689 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 PMID:21371258 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 PMID:24438969 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 PMID:20118908 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 PMID:18976417 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 PMID:16166283 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 PMID:24841586 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 PMID:20568590 AUTHORS Galper SL, Smith BD, Wilson LD TITLE Diagnosis and management of mycosis fungoides. JOURNAL Oncology (Williston Park) 24:491-501 (2010) |