What are the stages of mycosis fungoides cutaneous T-cell lymphoma (CTCL)?

Updated: Aug 15, 2018
  • Author: Lauren C Pinter-Brown, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Despite the fact that mycosis fungoides and Sézary syndrome are types of non-Hodgkin lymphoma, an entirely different staging system is used for these disorders. The system is based on the particular skin findings and findings of extracutaneous disease that are observed in affected patients, with the stages defined as follows:

  • Stage IA disease (as defined by the tumor, node, metastases, blood [TNMB] system) - Patchy or plaquelike skin disease involving less than 10% of the skin surface area (T1 skin disease)

  • Stage IB - Patchy or plaquelike skin disease involving 10% or more of the skin surface area (T2 skin disease)

  • Stage IIB disease - Tumors are present (T3 skin lesions)

  • Stage III disease - Generalized erythroderma is present

  • Stage IVA1 disease - Erythroderma and significant blood involvement occur [5]

  • Stage IVA2 disease - Lymph node biopsy result shows total effacement by atypical cells (LN4 node) [5]

  • Stage IVB disease - Visceral involvement (eg, liver, lung, bone marrow) occurs

These definitions are denoted in Table 3. Table 4 lists the TNMB stage definitions. Table 5 is a comparison of the 2 systems.

Table 3. Staging of Cutaneous T-Cell Lymphoma (Open Table in a new window)

Stage

Skin Lesions

Lymphadenopathy

Erythroderma

Histology

Patches/

plaques

(less than10%)

Plaques/

plaques

(10% or more)

Tumors

Lymph Nodes

Viscera

IA

+

-

-

-

-

-

-

IB

+ or -

+

-

-

-

-

-

IIA

+ or -

+ or -

+ or -

+

-

-

-

IIB

+ or -

+ or -

+

+ or -

-

-

-

III

+ or -

+ or -

+ or -

+ or -

+

-

-

IVA

+ or -

+ or -

+ or -

+ or -

+ or -

+

-

IVB

+ or -

+ or -

+ or -

+ or -

+ or -

+ or -

+

Table 4. TNMB Staging of Cutaneous T-Cell Lymphoma (Open Table in a new window)

Stage Class

Stage

Definition

T

(tumor)

T1

Patches/plaques involving less than 10% of body surface

T2

Patches/plaques involving 10% or more of body surface

T3

Tumor(s) present on skin

T4

Erythroderma

N

(nodes)

N0

No enlarged lymph node present

N1

Enlarged lymph nodes, histologically uninvolved

N2

No enlarged lymph node; 1 or more nodes histologically involved*

N3

Enlarged lymph nodes, histologically involved

M

(metastasis to viscera)

M0

No visceral lesion present

M1

Visceral involvement

B

(blood involvement)

B0

Circulating atypical lymphocytes (Sézary cells) less than 5% of lymphocytes

B1

Circulating atypical lymphocytes 5% or more of lymphocytes (Sézary syndrome)

*Uncommon finding, usually not considered/investigated.

 

Table 5. Comparison of Staging Systems for CTCL (Open Table in a new window)

Clinical Stage

TNM (B) Stage

IA

T1 N0 M0

IIB

T2 N0 M0

IIA

T1 N1 M0

T2 N1 M0

 

IIB

T3 N0 M0

T3 N1 M0

 

III

T4 N0 M0

T4 N1 M0

 

IVA

T1 N2 M0

T2 N2 M0

T3 N2 M0

T4 N2 M0

T1 N3 M0

T2 N3 M0

T3 N3 M0

T4 N3 M0

IVB

T1 N0 M1

T2 N0 M1

T3 N0 M1

T4 N0 M1

T1 N1 M1

T2 N1 M1

T3 N1 M1

T4 N1 M1

T1 N2 M1

T2 N2 M1

T3 N2 M1

T4 N2 M1

T1 N3 M1

T2 N3 M1

T3 N3 M1

T4 N3 M1


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