ISCL/USCLC/EORTC modified staging of PCLs
MF/SS . | Non-MF/SS CTCL and CBCL . | |||||||
---|---|---|---|---|---|---|---|---|
Skin (T) | T0* | Absence of clinically suspicious lesions | T0* | Absence of clinically suspicious lesions | Size/location of lesions† | |||
T1 | Patches, plaques, or papules <10% BSA | T1A | Patch only lesions | T1 | Solitary lesion | T1A | Solitary lesion <5 cm diameter | |
T1B | Plaque/papule+/− patch lesions | T1B | Solitary lesion ≥5 cm diameter | |||||
T2 | Patches, plaques, or papules ≥10% BSA | T2A | Patch only lesions | T2 | Multiple lesions limited to 1 body region or 2 contiguous body regions | T2A | All disease encompassing a <15 cm diameter circular area | |
T2B | Plaque/papule+/− patch lesions | T2B | All disease encompassing a 15 to <30 cm diameter circular area | |||||
T2C | All disease encompassing a ≥30 cm diameter circular area | |||||||
T3 | One or more tumors ≥1 cm diameter | T3 | Generalized skin involvement | T3A | Multiple lesions involving 2 noncontiguous body regions | |||
T3B | Multiple lesions involving ≥3 body regions | |||||||
T4 | Confluence of erythema covering ≥80% BSA† | |||||||
Nodes (N)‡ | N0 | No clinically abnormal LN; no biopsy necessary | N0 | No clinical or pathologic LN involvement | ||||
N1 | N1A | Pathology Dutch grade 1 or NCI LN 0-2: clone negative or equivocal | N1 | Involvement of 1 peripheral LN region that drains an area of current or prior skin involvement‖: biopsy positive for lymphoma | ||||
N1B | Pathology Dutch grade 1 or NCI LN 0-2: clone positive and identical to skin | |||||||
N2 | N2A | Dutch grade 2, NCI LN3: clone negative or equivocal | N2 | Involvement of > 2 peripheral LN regions or involvement of any LN region that does not drain in an area of current or prior skin involvement: biopsy positive for lymphoma | ||||
N2B | Dutch grade 2, NCI LN3: clone positive and identical to skin | |||||||
N3‡ (lymphoma) | N3A | Dutch grade 3-4, NCI LN4: clone negative or equivocal | N3 | Involvement of central LN: biopsy positive for lymphoma | ||||
N3B | Dutch grade 3-4, NCI LN4: clone positive and identical to skin | |||||||
NX | Clinically abnormal peripheral or central lymph node but no pathologic determination of representative LN. Other surrogate means of determining involvement may be determined by Tri-Society consensus | Nx | Clinically abnormal peripheral or central LN but no pathologic determination. Other surrogate means of determining involvement may be determined by Tri-Society consensus | |||||
Viscera (M) | M0 | No visceral involvement | M0 | No visceral involvement | ||||
M1a | BM only involvement | Clone positive and identical to skin | M1 | Visceral involvement | ||||
Clone negative or indeterminate | ||||||||
M1b | Non-BM visceral involvement | Clone positive and identical to skin | ||||||
Clone negative or indeterminate | ||||||||
Mx | Visceral involvement is neither confirmed nor refuted by available pathologic or imaging assessment | Mx | Visceral involvement is neither confirmed nor refuted by available pathologic or imaging assessment | |||||
Blood (B)§ | B0 | B0A | Clone negative or equivocal | Absence of significant blood involvement | ||||
B0B | Clone positive and identical to skin | |||||||
B1 | B1A | Clone negative or equivocal | Low blood tumor burden | |||||
B1B | Clone positive and identical to skin | |||||||
B2 | B2A | Clone negative or equivocal | High blood tumor burden | |||||
B2B | Clone positive and identical to skin | |||||||
Bx | BxA | Clone negative or equivocal | Unable to quantify blood involvement according to agreed upon guidelines | |||||
BxB | Clone positive and identical to skin |
MF/SS . | Non-MF/SS CTCL and CBCL . | |||||||
---|---|---|---|---|---|---|---|---|
Skin (T) | T0* | Absence of clinically suspicious lesions | T0* | Absence of clinically suspicious lesions | Size/location of lesions† | |||
T1 | Patches, plaques, or papules <10% BSA | T1A | Patch only lesions | T1 | Solitary lesion | T1A | Solitary lesion <5 cm diameter | |
T1B | Plaque/papule+/− patch lesions | T1B | Solitary lesion ≥5 cm diameter | |||||
T2 | Patches, plaques, or papules ≥10% BSA | T2A | Patch only lesions | T2 | Multiple lesions limited to 1 body region or 2 contiguous body regions | T2A | All disease encompassing a <15 cm diameter circular area | |
T2B | Plaque/papule+/− patch lesions | T2B | All disease encompassing a 15 to <30 cm diameter circular area | |||||
T2C | All disease encompassing a ≥30 cm diameter circular area | |||||||
T3 | One or more tumors ≥1 cm diameter | T3 | Generalized skin involvement | T3A | Multiple lesions involving 2 noncontiguous body regions | |||
T3B | Multiple lesions involving ≥3 body regions | |||||||
T4 | Confluence of erythema covering ≥80% BSA† | |||||||
Nodes (N)‡ | N0 | No clinically abnormal LN; no biopsy necessary | N0 | No clinical or pathologic LN involvement | ||||
N1 | N1A | Pathology Dutch grade 1 or NCI LN 0-2: clone negative or equivocal | N1 | Involvement of 1 peripheral LN region that drains an area of current or prior skin involvement‖: biopsy positive for lymphoma | ||||
N1B | Pathology Dutch grade 1 or NCI LN 0-2: clone positive and identical to skin | |||||||
N2 | N2A | Dutch grade 2, NCI LN3: clone negative or equivocal | N2 | Involvement of > 2 peripheral LN regions or involvement of any LN region that does not drain in an area of current or prior skin involvement: biopsy positive for lymphoma | ||||
N2B | Dutch grade 2, NCI LN3: clone positive and identical to skin | |||||||
N3‡ (lymphoma) | N3A | Dutch grade 3-4, NCI LN4: clone negative or equivocal | N3 | Involvement of central LN: biopsy positive for lymphoma | ||||
N3B | Dutch grade 3-4, NCI LN4: clone positive and identical to skin | |||||||
NX | Clinically abnormal peripheral or central lymph node but no pathologic determination of representative LN. Other surrogate means of determining involvement may be determined by Tri-Society consensus | Nx | Clinically abnormal peripheral or central LN but no pathologic determination. Other surrogate means of determining involvement may be determined by Tri-Society consensus | |||||
Viscera (M) | M0 | No visceral involvement | M0 | No visceral involvement | ||||
M1a | BM only involvement | Clone positive and identical to skin | M1 | Visceral involvement | ||||
Clone negative or indeterminate | ||||||||
M1b | Non-BM visceral involvement | Clone positive and identical to skin | ||||||
Clone negative or indeterminate | ||||||||
Mx | Visceral involvement is neither confirmed nor refuted by available pathologic or imaging assessment | Mx | Visceral involvement is neither confirmed nor refuted by available pathologic or imaging assessment | |||||
Blood (B)§ | B0 | B0A | Clone negative or equivocal | Absence of significant blood involvement | ||||
B0B | Clone positive and identical to skin | |||||||
B1 | B1A | Clone negative or equivocal | Low blood tumor burden | |||||
B1B | Clone positive and identical to skin | |||||||
B2 | B2A | Clone negative or equivocal | High blood tumor burden | |||||
B2B | Clone positive and identical to skin | |||||||
Bx | BxA | Clone negative or equivocal | Unable to quantify blood involvement according to agreed upon guidelines | |||||
BxB | Clone positive and identical to skin |
Based on Olsen et al, Olsen et al, and Kim et al.7-9 Changes or confirmation of staging are noted in bold in table above and in further description below. Options for characterizing clonality further by designation as A (clone negative or equivocal) and B (clone positive and identical to skin) are presented. If a clone in LN or viscera is detected but different from that identified in the skin, another concurrent lymphoproliferative process should be considered.
T0 is used for clinical trials in order to track clearance of lesions in the skin compartment. No patient with PCL at time of diagnosis should be T0.
Patients with both erythroderma and tumors may be designated as T4(T3).7 The BSA of 80% is used to define erythroderma in MF/SS at study entry, but any decrease in BSA during the study does not affect the entry classification.
Abnormal LNs are those now > 1.5 cm LDi according to the Lugano classification and confirmed by imaging. The pathological findings of a representative abnormal LN may apply to all abnormal lymph nodes.
Blood staging for MF/SS is defined currently as B0 = <250/μL of CD4+/CD26− or CD4+/CD7− cells, B1= does not meet criteria for B0 or B2, and B2 = ≥1000/μL of CD4+/CD26−or CD4+/CD7−cells or other aberrant population of lymphocytes identified by flow cytometry. It is expected that patients with high blood tumor burden (B2) will have a clone in the blood that is identical to that in the skin. Nonidentical T-cell clones are often detected in peripheral blood with increasing age and are of unknown clinical significance.
See Figure 2 for peripheral LN drainage areas of the body.