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Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®)

  • Last Modified: 01/24/2014

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Stage Information for Mycosis Fungoides and the Sézary Syndrome

Definitions of TNM

The stages that follow are defined by TNM classification. Peripheral blood involvement with mycosis fungoides or Sézary syndrome (MF/SS) cells is correlated with more advanced skin stage, lymph node and visceral involvement, and shortened survival.

MF/SS have a formal staging system proposed by the International Society for Cutaneous Lymphomas (ISCL) and the European Organization of Research and Treatment of Cancer (EORTC).[1,2]

Definitions of TNM

The American Joint Committee on Cancer (AJCC) has designated staging by TNM classification to define MF.[3]

Table 1. ISCL/EORTC Revision to the Classification of Mycosis Fungoides and the Sézary Syndromea
Skin  
T1Limited patches,b papules, and/or plaquesc covering <10% of the skin surface. May further stratify into T1a (patch only) vs. T1b (plaque ± patch).
T2Patches, papules, or plaques covering ≥10% of the skin surface. May further stratify into T2a (patch only) vs. T2b (plaque ± patch).
T3≥1 tumord (≥1 cm diameter).
T4Confluence of erythema covering ≥80% of body surface area.
Node
N0No clinically abnormal peripheral lymph nodes;e biopsy not required.
N1Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0–2.
N1aClone negative.f
N1bClone positive.f
N2Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.
N2aClone negative.f
N2bClone positive.f
N3Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3–4 or NCI LN4; clone positive or negative.
NxClinically abnormal peripheral lymph nodes; no histologic confirmation.
Visceral
M0No visceral organ involvement.
M1Visceral involvement (must have pathology confirmation,g and organ involved should be specified).
Peripheral Blood Involvement
B0Absence of significant blood involvement: ≤5% of peripheral blood lymphocytes are atypical (Sézary) cells.h
B0aClone negative.f
B0bClone positive.f
B1Low blood-tumor burden: >5% of peripheral blood lymphocytes are atypical (Sézary) cells but does not meet the criteria of B2.
B1aClone negative.f
B1bClone positive.f
B2High blood-tumor burden: ≥1,000/μL Sézary cellsh with positive clone.f

EORTC = European Organization of Research and Treatment of Cancer; ISCL = International Society for Cutaneous Lymphomas; NCI = National Cancer Institute.
aReprinted with permission from AJCC: Primary cutaneous lymphomas. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, p 613-5.
bFor skin, patch indicates any size skin lesion without significant elevation or induration. Presence/absence of hypo- or hyperpigmentation, scale, crusting, and/or poikiloderma should be noted.
cFor skin, plaque indicates any size skin lesion that is elevated or indurated. Presence or absence of scale, crusting, and/or poikiloderma should be noted. Histologic features such as folliculotropism or large-cell transformation (>25% large cells), CD30+ or CD30-, and clinical features, such as ulceration, are important to document.
dFor skin, tumor indicates at least 1 cm diameter solid or nodular lesion with evidence of depth and/or vertical growth. Note total number of lesions, total volume of lesions, largest size lesion, and region of body involved. Also, note if histologic evidence of large cell transformation has occurred. Phenotyping for CD30 is encouraged.
eFor node, abnormal peripheral lymph node(s) indicates any palpable peripheral node that on physical examination is firm, irregular, clustered, fixed, or ≥1.5 cm in diameter. Node groups examined on physical examination include: cervical, supraclavicular, epitrochlear, axillary, and inguinal. Central nodes, which are not generally amenable to pathologic assessment, are not currently considered in the nodal classification unless used to establish N3 histopathologically.
fA T-cell clone is defined by polymerase chain reaction or Southern blot analysis of the T-cell receptor (TCR) gene.
gFor viscera, spleen and liver may be diagnosed by imaging criteria.
hFor blood, Sézary cells are defined as lymphocytes with hyper-convoluted cerebriform nuclei. If Sézary cells are not able to be used to determine tumor burden for B2, then one of the following modified ISCL criteria along with a positive clonal rearrangement of the TCR may be used instead: (1) expanded CD4+ or CD3+ cells with CD4/CD8 ratio of ≥10; and (2) expanded CD4+ cells with abnormal immunophenotype, including loss of CD7 or CD26.

Table 2. Anatomic Stage/Prognostic Groupsa,b
ISCL/EORTC Revision to the Staging of Mycosis Fungoides and the Sézary Syndrome 
Stage T N M Peripheral Blood Involvement 
IA1000, 1
IB2000, 1
IIA1, 21, 200, 1
IIB30–200, 1
III40–200, 1
IIIA40–200
IIIB40–201
IVA11–40–202
IVA21–4300–2
IVB1–40–310–2

EORTC = European Organization of Research and Treatment of Cancer; ISCL = International Society for Cutaneous Lymphomas.
aReprinted with permission from AJCC: Primary cutaneous lymphomas. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, p 613-5.
bAdapted from Olsen et al.[1]

References
  1. 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 110 (6): 1713-22, 2007.  [PUBMED Abstract]

  2. 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 28 (31): 4730-9, 2010.  [PUBMED Abstract]

  3. Primary cutaneous lymphomas. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 614-5.