Stage Information

TNM(B) Definitions
TNM Stage Groupings

Note: The American Joint Committee on Cancer has recently published a new edition of the AJCC Cancer Staging Manual, which includes revisions to the staging for this disease. The PDQ Adult Treatment Editorial Board, which is responsible for maintaining this summary, is currently reviewing the new staging to determine the changes that need to be made in the summary. In addition to updating this Stage Information section, additional changes may need to be made to other parts of this summary to ensure that it is up-to-date. The changes will be made as soon as possible.

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]

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)

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.