Mycosis Fungoides: Assessment and Management
Abstract
Mycosis fungoides (MF) is a type of non-Hodgkin lymphoma that primarily affects mature T-cells and initially presents as skin lesions, but can potentially involve the lymph nodes, blood and, in rare cases, internal organs. It is the most common type of primary cutaneous T-cell lymphoma. The skin lesions in MF can appear as patches or plaques that are either confined to a particular area or are spread over a large area. In addition to these lesions, MF can present as tumors or erythroderma.1 MF is distinct from other primary cutaneous T-cell lymphomas (CTCLs) due to its distinct clinical features and histopathology. This article will focus on the clinical features, diagnosis, staging, and treatment of MF. While another subtype of CTCL, known as Sézary syndrome, also exists, it is beyond the scope of the present discussion.
References
Hodak E, Amitay-Laish I. Mycosis fungoides: A great imitator. Clinics in Dermatology. 2019 May 1;37(3):255-67. doi:10.1016/j.clindermatol.2019.01.004.
Ungewickell A, Bhaduri A, Rios E, Reuter J, Lee CS, Mah A, Zehnder A, Ohgami R, Kulkarni S, Armstrong R, Weng WK. Genomic analysis of mycosis fungoides and Sézary syndrome identifies recurrent alterations in TNFR2. Nature Genetics. 2015 Sep;47(9):1056-60. doi:10.1038/ng.3370.
Whittemore AS, Holly EA, Lee IM, Abel EA, Adams RM, Nickoloff BJ, Bley L, Peters JM, Gibney C. Mycosis fungoides in relation to environmental exposures and immune response: a case-control study. JNCI: Journal of the National Cancer Institute. 1989 Oct 18;81(20):1560-7. doi:10.1093/jnci/81.20.1560
Korgavkar K, Xiong M, Weinstock M. Changing incidence trends of cutaneous T-cell lymphoma. JAMA dermatology. 2013 Nov 1;149(11):1295-9. doi:10.1001/jamadermatol.2013.5526.
Sant M, Allemani C, Tereanu C, De Angelis R, Capocaccia R, Visser O, Marcos-Gragera R, Maynadié M, Simonetti A, Lutz JM, Berrino F. Incidence of hematologic malignancies in Europe by morphologic subtype: results of the HAEMACARE project. Blood, The Journal of the American Society of Hematology. 2010 Nov 11;116(19):3724-34. doi:10.1182/blood-2010-05-282632.
Sun G, Berthelot C, Li Y, Glass II DA, George D, Pandya A, Kurzrock R, Duvic M. Poor prognosis in non-Caucasian patients with early-onset mycosis fungoides. Journal of the American Academy of Dermatology. 2009 Feb 1;60(2):231-5. doi:10.1016/j.jaad.2008.09.063.
Hodak E, Klein T, Gabay B, Ben-Amitai D, Bergman R, Gdalevich M, Feinmesser M, Maron L, David M. Familial mycosis fungoides: report of 6 kindreds and a study of the HLA system. Journal of the American Academy of Dermatology. 2005 Mar 1;52(3):393-402. doi:10.1016/j.jaad.2003.12.052.
Zackheim HS, McCalmont TH. Mycosis fungoides: the great imitator. Journal of the American Academy of Dermatology. 2002 Dec 1;47(6):914-8. doi:10.1067/mjd.2002.124696.
Pimpinelli N, Olsen EA, Santucci M, Vonderheid E, Haeffner AC, Stevens S, Burg G, Cerroni L, Dreno B, Glusac E, Guitart J. Defining early mycosis fungoides. Journal of the American Academy of Dermatology. 2005 Dec 1;53(6):1053-63. doi:10.1016/j.jaad.2005.08.057.
Morales MM, Olsen J, Johansen P, Kaerlev L, Guénel P, Arveux P, Wingren G, Hardell L, Ahrens W, Stang A, Llopis A. Viral infection, atopy and mycosis fungoides: a European multicentre case–control study. European Journal of Cancer. 2003 Mar 1;39(4):511-6. doi:10.1016/s0959-8049(02)00773-6.
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, Ralfkiaer E, Chimenti S, Diaz-Perez JL, Duncan LM, Grange F. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005 May 15;105(10):3768-85. doi:10.1182/blood-2004-09-3502.
Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, Jaffe ES. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood, The Journal of the American Society of Hematology. 2019 Apr 18;133(16):1703-14. doi:10.1182/blood-2018-11-881268.
Fung MA, Murphy MJ, Hoss DM, Grant-Kels JM. Practical evaluation and management of cutaneous lymphoma. Journal of the American Academy of Dermatology. 2002 Mar 1;46(3):325-60. doi:10.1067/mjd.2002.121355.
Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, Zackheim H, Duvic M, Estrach T, Lamberg S, Wood G. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood, The Journal of the American Society of Hematology. 2007 Sep 15;110(6):1713-22. doi:10.1182/blood-2007-03-055749.
Duvic M, Lemak NA, Redman JR, Eifel PJ, Tucker SL, Cabanillas FF, Kurzrock R. Combined modality therapy for cutaneous T-cell lymphoma. Journal of the American Academy of Dermatology. 1996 Jun 1;34(6):1022-9. doi:10.1016/s0190-9622(96)90282-0.
Quaglino P, Prince HM, Cowan R, Vermeer M, Papadavid E, Bagot M, Servitjie O, Berti E, Guenova E, Stadler R, Querfeld C. Treatment of early-stage mycosis fungoides: results from the PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study. British Journal of Dermatology. 2021 Apr 1;184(4):722-30. doi:10.1111/bjd.19252.
Thomsen KR, Hammar HA, Molin LA, Volden G. Retinoids plus PUVA (RePUVA) and PUVA in mycosis fungoides, plaque stage. A report from the Scandinavian Mycosis Fungoides Group. Acta Dermato-venereologica. 1989 Nov 17;69(6):536-8.
Kuzel TM, Roenigk Jr HH, Samuelson E, Herrmann JJ, Hurria A, Rademaker AW, Rosen ST. Effectiveness of interferon alfa-2a combined with phototherapy for mycosis fungoides and the Sézary syndrome. Journal of Clinical Oncology. 1995 Jan;13(1):257-63. doi:10.1200/JCO.1995.13.1.257.
Jennings T, Duffy R, Gochoco A, Knoblauch K, Shi W, Alpdogan SO, Porcu P, Werner-Wasik M, Sahu J. Valchlor maintenance therapy for patients with mycosis fungoides who received low dose total skin electron beam treatment. Chin Clin Oncol. 2019 Feb 1;8(1):13. doi:10.21037/cco.2018.10.01.
Kaye FJ, Bunn Jr PA, Steinberg SM, Stocker JL, Ihde DC, Fischmann AB, Glatstein EJ, Schechter GP, Phelps RM, Foss FM, Parlette III HL. A randomized trial comparing combination electron-beam radiation and chemotherapy with topical therapy in the initial treatment of mycosis fungoides. New England Journal of Medicine. 1989 Dec 28;321(26):1784-90. doi:10.1056/NEJM198912283212603.
Chinn DM, Chow S, Kim YH, Hoppe RT. Total skin electron beam therapy with or without adjuvant topical nitrogen mustard or nitrogen mustard alone as initial treatment of T2 and T3 mycosis fungoides. International Journal of Radiation Oncology* Biology* Physics. 1999 Mar 15;43(5):951-8. doi:10.1016/s0360-3016(98)00517-3.
Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. Journal of the American Academy of Dermatology. 2014 Feb 1;70(2):205-e1. doi:10.1016/j.jaad.2013.08.033.
Hughes CF, Khot A, McCormack C, Lade S, Westerman DA, Twigger R, Buelens O, Newland K, Tam C, Dickinson M, Ryan G. Lack of durable disease control with chemotherapy for mycosis fungoides and Sézary syndrome: a comparative study of systemic therapy. Blood, The Journal of the American Society of Hematology. 2015 Jan 1;125(1):71-81. doi:10.1182/blood-2014-07-588236.
Grozea PN, Jones SE, McKelvey EM, Coltman Jr CA, Fisher R, Haskins CL. Combination chemotherapy for mycosis fungoides: a Southwest Oncology Group study. Cancer Treatment Reports. 1979 Apr 1;63(4):647-53.
Prince HM, Duvic M, Martin A, Sterry W, Assaf C, Straus DJ. Incidence of spontaneous remission in patients with CD25-positive mycosis fungoides/Sezary syndrome receiving placebo. Journal of the American Academy of Dermatology. 2012 Nov 1;67(5):867-75. doi:10.1016/j.jaad.2011.12.027.
