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TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sézary 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)

Youn H. Kim, Rein Willemze, Nicola Pimpinelli, Sean Whittaker, Elise A. Olsen, Annamari Ranki, Reinhard Dummer and Richard T. Hoppe

Data supplements

Article Figures & Data

Figures

  • Figure 1

    Body regions as defined in the proposed TNM system for the designation of T (skin involvement) category. Left and right extremities are assessed as separate body regions. The designation of these body regions are based on regional LN drainage patterns.

  • Figure 2

    Clinical examples of proposed T1 (discrete solitary lesion) category of primary cutaneous lymphomas. (A) T1a (solitary lesion equal or less than 5 cm), CD30 + anaplastic large cell lymphoma. (B) T1b (solitary lesion greater than 5 cm), NK/T-cell, nasal-type, lymphoma.

  • Figure 3

    Clinical examples of proposed T2a (regional involvement with lesions within a 15 cm diameter area) category of primary cutaneous lymphomas. (A) CD30 + anaplastic large cell lymphoma. (B) Follicle center lymphoma. (C) Diffuse large B-cell lymphoma, leg-type.

  • Figure 4

    Clinical examples of proposed T2b (regional involvement with lesions in an area between 15 cm and 30 cm diameter) category of primary cutaneous lymphomas. (A) diffuse large B-cell lymphoma, leg-type. (B) Follicle center lymphoma. (C) Follicle center lymphoma.

  • Figure 5

    Clinical examples of proposed T2c (regional involvement with lesions more than 30 cm in diameter) category of cutaneous lymphomas. (A) Diffuse large B-cell lymphoma, leg-type. (B) Follicle center lymphoma. (C) Diffuse large B-cell lymphoma, leg-type.

  • Figure 6

    Clinical examples of proposed T3 (generalized) category of cutaneous lymphomas. (A) T3a (multiple lesions involving 2 noncontiguous body regions), follicle center lymphoma. (B) T3b (multiple lesions involving 3 or more body regions), CD30 + anaplastic large-cell lymphoma.

Tables

  • Table 1

    WHO-EORTC classification of primary cutaneous lymphomas other than mycosis fungoides and Sézary syndrome

    Cutaneous T-cell and NK-cell lymphomas
        Primary cutaneous CD30+ lymphoproliferative disorders
        Primary cutaneous anaplastic large-cell lymphoma
        Subcutaneous panniculitis-like T-cell lymphoma
        Extranodal NK/T-cell lymphoma, nasal-type
        Primary cutaneous peripheral T-cell lymphoma, unspecified
            Primary cutaneous aggressive epidermotrophic CD8+ T-cell lymphoma, provisional
            Cutaneous γ/δ T-cell lymphoma, provisional
            Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma, provisional
            Primary cutaneous peripheral T-cell lymphoma, unspecified, other
    Cutaneous B-cell lymphomas
        Primary cutaneous marginal zone B-cell lymphoma
        Primary cutaneous follicle center lymphoma
        Primary cutaneous diffuse large B-cell lymphoma, leg-type
        Primary cutaneous diffuse large B-cell lymphoma, other
        Intravascular large B-cell lymphoma
    Precursor hematologic neoplasm: CD4+/CD56+ hematodermic neoplasm, blastic NK-cell lymphoma
  • Table 2

    ISCL/EORTC proposal on TNM classification of cutaneous lymphoma other than MF/SS

    Classification
    T
        T1: Solitary skin involvement
            T1a: a solitary lesion <5 cm diameter
            T1b: a solitary >5 cm diameter
        T2: Regional skin involvement: multiple lesions limited to 1 body region or 2 contiguous body regions*
            T2a: all-disease-encompassing in a <15-cm-diameter circular area
            T2b: all-disease-encompassing in a >15- and <30-cm-diameter circular area
            T2c: all-disease-encompassing in a >30-cm-diameter circular area
        T3: Generalized skin involvement
            T3a: multiple lesions involving 2 noncontiguous body regions
            T3b: multiple lesions involving ≥3 body regions
    N
        N0: No clinical or pathologic lymph node involvement
        N1: Involvement of 1 peripheral lymph node region that drains an area of current or prior skin involvement
        N2: Involvement of 2 or more peripheral lymph node regions or involvement of any lymph node region that does not drain an area of current or prior skin involvement
        N3: Involvement of central lymph nodes
    M
        M0: No evidence of extracutaneous non–lymph node disease
        M1: Extracutaneous non–lymph node disease present
    • * Definition of body regions (see Figure 1): Head and neck: inferior border—superior border of clavicles, T1 spinous process. Chest: superior border—superior border of clavicles; inferior border—inferior margin of rib cage; lateral borders—mid-axillary lines, glenohumeral joints (inclusive of axillae). Abdomen/genital: superior border—inferior margin of rib cage; inferior border—inguinal folds, anterior perineum; lateral borders—mid-axillary lines. Upper back: superior border—T1 spinous process; inferior border—inferior margin of rib cage; lateral borders—mid-axillary lines. Lower back/buttocks: superior border—inferior margin of rib cage; inferior border—inferior gluteal fold, anterior perineum (inclusive of perineum); lateral borders—mid-axillary lines. Each upper arm: superior borders—glenohumeral joints (exclusive of axillae); inferior borders—ulnar/radial-humeral (elbow) joint. Each lower arm/hand: superior borders—ulnar/radial-humeral (elbow) joint. Each upper leg (thigh): superior borders—inguinal folds, inferior gluteal folds; inferior borders—mid-patellae, mid-popliteal fossae. Each lower leg/foot: superior borders—mid-patellae, mid-popliteal fossae.

    • Definition of lymph node regions is consistent with the Ann Arbor system: Peripheral sites: antecubital, cervical, supraclavicular, axillary, inguinal-femoral, and popliteal. Central sites: mediastinal, pulmonary hilar, paraortic, iliac.

  • Table 3

    ISCL/EORTC recommendations for staging evaluation in cutaneous lymphomas other than MF/SS

    Complete history/review of systems and physical examination
    Laboratory studies
        Complete blood count, comprehensive serum chemistries, serum LDH
        Whenever indicated, relevant flow cytometric studies of peripheral blood mononuclear cells
    Imaging studies*
        CT of chest, abdomen and pelvis with contrast alone or with whole-body PET (18F-FDG); include CT or ultrasound of neck if clinically indicated
        Whole-body integrated PET/CT (as alternative imaging study to the standard contrast-enhanced CT)
    Bone marrow biopsy and aspirate
        Required in cutaneous lymphomas with intermediate to aggressive clinical behavior as categorized in the WHO-EORTC classification
        Should be considered in cutaneous lymphomas with indolent clinical behavior, but not required unless indicated by other staging assessments
    Additional studies as indicated clinically
    • * Lymph nodes that are >1.0 cm in short axis and/or have significantly increased PET activity should be sampled for tissue examination (an excisional biopsy is preferable whenever possible).

    • At the time of this proposal, there is not a unified standard for bone marrow examination as part of the staging evaluation in cutaneous lymphomas with indolent clinical behavior. The clinician should follow the standard of care of his or her regional practice.