Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

Lymphoma - Non B cell neoplasms

T/NK cell disorders

Primary cutaneous lymphoma - staging


Reviewer: Dragos Luca, M.D. (see Reviewers page)
Revised: 27 October 2011, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

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

Notes:
● 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

Notes:
● 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 (SÚzary) cells
B0a: Clone negative
B0b: Clone positive
B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (SÚzary) cells but does not meet the criteria of B2
B1a: Clone negative
B1b: Clone positive
B2: High blood tumor burden: 1000/μL SÚzary cells or more with positive clone

Notes:
● SÚzary cells are defined as lymphocytes with hyperconvoluted cerebriform nuclei. If SÚzary 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
=========================================================================

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
=========================================================================

References: Blood 2007;110:1713 (revisions to staging), erratum in: Blood 2008;111:4830

End of Lymphoma - Non B cell neoplasms > T/NK cell disorders > Primary cutaneous lymphoma-staging


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).