Lymphoma and plasma cell neoplasms
T / NK cell disorders
Primary cutaneous lymphoma - staging

Author: Dragos Luca, M.D. (see Authors page)

Revised: 28 March 2017, last major update August 2011

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: staging [title] Primary cutaneous lymphoma

Cite this page: Primary cutaneous lymphoma - staging. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonBstagingprimarycutaneous.html. Accessed August 18th, 2017.
Skin
  • T1: Limited patches, papules and / or plaques covering less than 10% of the skin surface. May further stratify into T1a (patch only) vs. T1b (plaque +/- patch)
  • T2: Patches, papules or plaques covering 10% or more of the skin surface. May further stratify into T2a (patch only) vs. T2b (plaque +/- patch)
  • T3: One or more tumors (1 cm or more in diameter)
  • T4: Confluence of erythema covering 80% or more of body surface area

Note:
  • Patch indicates any size skin lesion without significant elevation or induration; presence / absence of hypo or hyperpigmentation, scale, crusting or poikiloderma should be noted
  • Plaque indicates any size skin lesion that is elevated or indurated; presence / absence of scale, crusting and / or poikiloderma should be noted; histologic features such as folliculotropism or large cell transformation ( > 25% large cells), CD30+ or CD30- and clinical features such as ulceration are important to document
  • Tumor indicates at least one 1 cm diameter solid or nodular lesion with evidence of depth and / or vertical growth; note total number of lesions, total volume of lesions, largest size lesion and region of body involved; also note if histologic evidence of large cell transformation has occurred; phenotyping for CD30 encouraged

Table
Node
  • N0: No clinically abnormal peripheral lymph nodes; biopsy not required
  • N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0 - 2
  • N1a: Clone negative
  • N1b: Clone positive
  • N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3
  • N2a: Clone negative
  • N2b: Clone positive
  • N3: Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3 - 4 or NCI LN4; clone positive or negative
  • Nx: Clinically abnormal peripheral lymph nodes; no histologic confirmation

Note:
  • Abnormal peripheral lymph node(s) indicates any palpable peripheral node that on physical examination is firm, irregular, clustered, fixed or 1.5 cm or larger in diameter. Node groups examined on physical examination include cervical, supraclavicular, epitrochlear, axillary and inguinal. Central nodes, which are not generally amenable to pathologic assessment, are not currently considered in the nodal classification unless used to establish N3 histopathologically
  • A T cell clone is defined by PCR or Southern blot analysis of the T cell receptor gene
  • Table: comparison of updated ISCL / EORTC, Dutch system and NCI-VA classification for nodes
Visceral
  • M0: No visceral organ involvement
  • M1: Visceral involvement (must have pathology confirmation and organ involved should be specified)

Note:
  • For viscera, spleen and liver may be diagnosed by imaging criteria
  • Blood
    • B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sézary) cells
    • B0a: Clone negative
    • B0b: Clone positive
    • B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sézary) cells but does not meet the criteria of B2
    • B1a: Clone negative
    • B1b: Clone positive
    • B2: High blood tumor burden: 1000 / μL Sézary cells or more with positive clone

    Note:
    • Sézary cells are defined as lymphocytes with hyperconvoluted cerebriform nuclei. If Sézary cells are not able to be used to determine tumor burden for B2, then one of the following modified ISCL criteria along with a positive clonal rearrangement of the TCR may be used instead:
      1. Expanded CD4+ or CD3+ cells with CD4 / CD8 ratio of 10 or more
      2. Expanded CD4+ cells with abnormal immunophenotype including loss of CD7 or CD26
    • A T cell clone is defined by PCR or Southern blot analysis of the T cell receptor gene
    Staging / staging classifications
    • Stage IA: T1 N0 M0 Peripheral blood: 0, 1
    • Stage IB: T2 N0 M0 Peripheral blood: 0, 1
    • Stage II: T1 - 2 N1 - 2 M0 Peripheral blood: 0, 1
    • Stage IIB: T3 N0 - 2 M0 Peripheral blood: 0, 1
    • Stage III: T4 N0 - 2 M0 Peripheral blood: 0, 1
    • Stage IIIA: T4 N0 - 2 M0 Peripheral blood: 0
    • Stage IIIB: T4 N0 - 2 M0 Peripheral blood: 1
    • Stage IVA1: T1 - 4 N0-2 M0 Peripheral blood: 2
    • Stage IVA2: T1 - 4 N3 M0 Peripheral blood: 0 - 2
    • Stage IVB: T1 - 4 & N0-3 M1 Peripheral blood: 0 - 2
    Additional references