Staging and prognosis of mycosis fungoides and Sézary syndrome
- Richard T Hoppe, MD
Richard T Hoppe, MD
- Professor of Radiation Oncology
- Stanford University School of Medicine
- Youn H Kim, MD
Youn H Kim, MD
- Professor of Dermatology
- Stanford University School of Medicine
- Section Editors
- Timothy M Kuzel, MD, FACP
Timothy M Kuzel, MD, FACP
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine, Rush University Medical Center
- Chief, Division of Hematology/Oncology/Cell Therapy
- John A Zic, MD
John A Zic, MD
- Section Editor — Cutaneous Lymphoma
- Associate Professor of Medicine/Dermatology
- Vanderbilt University School of Medicine
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T cell lymphoma (CTCL).
●MF is a mature T cell non-Hodgkin lymphoma with presentation in the skin but with potential involvement of the nodes, blood, and viscera. Skin lesions include patches or plaques that may be localized or widespread, tumors, and erythroderma.
●SS is a distinctive erythrodermic CTCL with a leukemic involvement of malignant T cells clonally matching that in the skin.
This group of disorders differs from other primary CTCLs by virtue of unique clinical features and histopathology. This topic review will discuss the staging, response criteria, and prognosis of MF and SS. The diagnosis and management of these disorders are discussed separately. (See "Clinical manifestations, pathologic features, and diagnosis of mycosis fungoides" and "Clinical presentation, pathologic features, and diagnosis of Sézary syndrome" and "Treatment of early stage (IA to IIA) mycosis fungoides" and "Treatment of advanced stage (IIB to IV) mycosis fungoides".)
TNMB criteria — The standard staging classification system for mycosis fungoides (MF) and Sézary syndrome (SS) is the TNMB system, which is based upon an evaluation of the skin (T), lymph nodes (N), visceral involvement (M), and blood (B) (table 1A-B) . The standard staging evaluation includes a careful examination of the skin (especially the scalp, palms, soles, and perineum) with skin biopsy, a complete blood count with Sézary cell analysis, screening chemistries including lactate dehydrogenase, and a chest x-ray (table 2). Lymph node biopsies, not fine needle aspirations, are obtained if lymphadenopathy is present, since lymph node involvement affects both the stage and prognosis.
- Olsen E, Vonderheid E, Pimpinelli N, et al. 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 2007; 110:1713.
- Olsen EA, Whittaker S, Kim YH, et al. Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer. J Clin Oncol 2011; 29:2598.
- Nagel TR, Schunk JE. Using the hand to estimate the surface area of a burn in children. Pediatr Emerg Care 1997; 13:254.
- Wachtel TL, Berry CC, Wachtel EE, Frank HA. The inter-rater reliability of estimating the size of burns from various burn area chart drawings. Burns 2000; 26:156.
- Lund CC, Browder NC. The estimation of areas of burns. Surg Gynecol Obstet 1944; 79:352.
- Hidvegi N, Nduka C, Myers S, Dziewulski P. Estimation of breast burn size. Plast Reconstr Surg 2004; 113:1591.
- Stevens SR, Ke MS, Parry EJ, et al. Quantifying skin disease burden in mycosis fungoides-type cutaneous T-cell lymphomas: the severity-weighted assessment tool (SWAT). Arch Dermatol 2002; 138:42.
- Duvic M, Martin AG, Kim Y, et al. Phase 2 and 3 clinical trial of oral bexarotene (Targretin capsules) for the treatment of refractory or persistent early-stage cutaneous T-cell lymphoma. Arch Dermatol 2001; 137:581.
- Hoppe RT, Wood GS, Abel EA. Mycosis fungoides and the Sézary syndrome: pathology, staging, and treatment. Curr Probl Cancer 1990; 14:293.
- Talpur R, Singh L, Daulat S, et al. Long-term outcomes of 1,263 patients with mycosis fungoides and Sézary syndrome from 1982 to 2009. Clin Cancer Res 2012; 18:5051.
- http://www.nccn.org/professionals/physician_gls/pdf/nhl.pdf (Accessed on May 02, 2012).
- Kulin PA, Marglin SI, Shuman WP, et al. Diagnostic imaging in the initial staging of mycosis fungoides and Sézary syndrome. Arch Dermatol 1990; 126:914.
- Tsai EY, Taur A, Espinosa L, et al. Staging accuracy in mycosis fungoides and sezary syndrome using integrated positron emission tomography and computed tomography. Arch Dermatol 2006; 142:577.
- Kamstrup MR, Gniadecki R, Friberg L. Integrated positron-emission tomography and computed tomography manifestations of cutaneous T-cell lymphoma. Arch Dermatol 2012; 148:1420.
- Battistella M, Sallé de Chou C, de Bazelaire C, et al. Lymph node image-guided core-needle biopsy for cutaneous T-cell lymphoma staging. Br J Dermatol 2016.
- Sausville EA, Worsham GF, Matthews MJ, et al. Histologic assessment of lymph nodes in mycosis fungoides/Sézary syndrome (cutaneous T-cell lymphoma): clinical correlations and prognostic import of a new classification system. Hum Pathol 1985; 16:1098.
- Salhany KE, Greer JP, Cousar JB, Collins RD. Marrow involvement in cutaneous T-cell lymphoma. A clinicopathologic study of 60 cases. Am J Clin Pathol 1989; 92:747.
- Scarisbrick JJ, Prince HM, Vermeer MH, et al. Cutaneous Lymphoma International Consortium Study of Outcome in Advanced Stages of Mycosis Fungoides and Sézary Syndrome: Effect of Specific Prognostic Markers on Survival and Development of a Prognostic Model. J Clin Oncol 2015; 33:3766.
- Kim YH, Bishop K, Varghese A, Hoppe RT. Prognostic factors in erythrodermic mycosis fungoides and the Sézary syndrome. Arch Dermatol 1995; 131:1003.
- Agar NS, Wedgeworth E, Crichton S, et al. Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal. J Clin Oncol 2010; 28:4730.
- Kim YH, Jensen RA, Watanabe GL, et al. Clinical stage IA (limited patch and plaque) mycosis fungoides. A long-term outcome analysis. Arch Dermatol 1996; 132:1309.
- Ally MS, Pawade J, Tanaka M, et al. Solitary mycosis fungoides: a distinct clinicopathologic entity with a good prognosis: a series of 15 cases and literature review. J Am Acad Dermatol 2012; 67:736.
- Kim YH, Chow S, Varghese A, Hoppe RT. Clinical characteristics and long-term outcome of patients with generalized patch and/or plaque (T2) mycosis fungoides. Arch Dermatol 1999; 135:26.
- Quaglino P, Pimpinelli N, Berti E, et al. Time course, clinical pathways, and long-term hazards risk trends of disease progression in patients with classic mycosis fungoides: a multicenter, retrospective follow-up study from the Italian Group of Cutaneous Lymphomas. Cancer 2012; 118:5830.
- de Coninck EC, Kim YH, Varghese A, Hoppe RT. Clinical characteristics and outcome of patients with extracutaneous mycosis fungoides. J Clin Oncol 2001; 19:779.
- Klemke CD, Dippel E, Assaf C, et al. Follicular mycosis fungoides. Br J Dermatol 1999; 141:137.
- Bonta MD, Tannous ZS, Demierre MF, et al. Rapidly progressing mycosis fungoides presenting as follicular mucinosis. J Am Acad Dermatol 2000; 43:635.
- Gilliam AC, Lessin SR, Wilson DM, Salhany KE. Folliculotropic mycosis fungoides with large-cell transformation presenting as dissecting cellulitis of the scalp. J Cutan Pathol 1997; 24:169.
- van Doorn R, Scheffer E, Willemze R. Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic and follow-up study of 51 patients. Arch Dermatol 2002; 138:191.
- Kim SY. Follicular mycosis fungoides. Am J Dermatopathol 1985; 7:300.
- Gerami P, Rosen S, Kuzel T, et al. Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma. Arch Dermatol 2008; 144:738.
- Lehman JS, Cook-Norris RH, Weed BR, et al. Folliculotropic mycosis fungoides: single-center study and systematic review. Arch Dermatol 2010; 146:607.
- Diamandidou E, Colome-Grimmer M, Fayad L, et al. Transformation of mycosis fungoides/Sezary syndrome: clinical characteristics and prognosis. Blood 1998; 92:1150.
- World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Swerdlow SH, Campo E, Harris NL (Eds), IARC Press, Lyon 2008.
- Kadin ME, Hughey LC, Wood GS. Large-cell transformation of mycosis fungoides-differential diagnosis with implications for clinical management: a consensus statement of the US Cutaneous Lymphoma Consortium. J Am Acad Dermatol 2014; 70:374.
- Herrmann JL, Hughey LC. Recognizing large-cell transformation of mycosis fungoides. J Am Acad Dermatol 2012; 67:665.
- Vergier B, de Muret A, Beylot-Barry M, et al. Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases. French Study Group of Cutaneious Lymphomas. Blood 2000; 95:2212.
- Arulogun SO, Prince HM, Ng J, et al. Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation. Blood 2008; 112:3082.
- Benner MF, Jansen PM, Vermeer MH, Willemze R. Prognostic factors in transformed mycosis fungoides: a retrospective analysis of 100 cases. Blood 2012; 119:1643.
- Talpur R, Sui D, Gangar P, et al. Retrospective Analysis of Prognostic Factors in 187 Cases of Transformed Mycosis Fungoides. Clin Lymphoma Myeloma Leuk 2016; 16:49.
- Ai WZ, Keegan TH, Press DJ, et al. Outcomes after diagnosis of mycosis fungoides and Sézary syndrome before 30 years of age: a population-based study. JAMA Dermatol 2014; 150:709.
- Benton EC, Crichton S, Talpur R, et al. A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. Eur J Cancer 2013; 49:2859.
- TNMB criteria
- Skin evaluation
- - Total body skin examination
- - Percent body surface area estimates
- - Disease severity estimates
- Peripheral blood
- Imaging studies
- Lymph node biopsy
- Bone marrow aspirate and biopsy
- Impact of disease stage
- Folliculotropic variant
- Transformation to large cell histology
- Younger age
- Prognostic indices
- RESPONSE ASSESSMENT
- RESPONSE CRITERIA
- SUMMARY AND RECOMMENDATIONS