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Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®)
Patient VersionHealth Professional VersionEn españolLast Modified: 05/22/2008



Purpose of This PDQ Summary






General Information






Cellular Classification







Stage Information






Treatment Option Overview






Stage I Mycosis Fungoides/Sézary Syndrome






Stage II Mycosis Fungoides/Sézary Syndrome






Stage III Mycosis Fungoides/Sézary Syndrome






Stage IV Mycosis Fungoides/Sézary Syndrome






Recurrent Mycosis Fungoides/Sézary Syndrome






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Changes to This Summary (05/22/2008)






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Stage Information

TNM(B) Definitions
TNM Stage Groupings

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 but probably provides no independent prognostic information beyond that associated with TNM staging. In a multivariate analysis, the two most important prognostic factors are the presence of visceral disease and type of skin involvement.

The American Joint Committee on Cancer (AJCC) has designated staging by TNM classification to define mycosis fungoides.[1]

TNM(B) Definitions

Primary tumor (T)

  • T1: Limited patch/plaque (<10% of skin surface involved)
  • T2: Generalized patch/plaque (≥10% of skin surface involved)
  • T3: Cutaneous tumors (one or more)
  • T4: Generalized erythroderma (with or without patches, plaques, or tumors)

 [Note: Pathology of T1–T4 is diagnostic of cutaneous T-cell lymphoma (CTCL). When characteristics of more than one T-type tumor exist, both are recorded and the highest is used for staging, for example, T4(3).]

Regional lymph nodes (N)

  • N0: Lymph nodes clinically uninvolved; the pathology is negative for CTCL
  • N1: Lymph nodes clinically enlarged, histologically uninvolved; the pathology is negative for CTCL
  • N2: Lymph nodes clinically unenlarged, histologically involved; the pathology is positive for CTCL
  • N3: Lymph nodes enlarged and histologically involved; the pathology is positive for CTCL

Distant metastasis (M)

  • M0: No visceral disease
  • M1: Visceral disease present

The TNM classification includes a subcategory for patients with CTCL:

Blood involvement (B)

  • B0: No circulating atypical cells (<1000 Sézary cells [CD4 + CD7-]/mL)
  • B1: Circulating atypical cells (≥1000 Sézary cells [CD4 + CD7-]/mL)
TNM Stage Groupings

Stage IA

  • T1, N0, M0

Stage IB

  • T2, N0, M0

Stage IIA

  • T1-T2, N1, M0

Stage IIB

  • T3, N0–N1, M0

Stage IIIA

  • T4, N0, M0

Stage IIIB

  • T4, N1, M0

Stage IVA

  • T1–T4, N2, M0
  • T1–T4, N3, M0

Stage IVB

  • T1–T4, N0, M1
  • T1–T4, N1, M1
  • T1–T4, N2, M1
  • T1–T4, N3, M1

References

  1. Lymphoid neoplasms. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 393-406. 

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