Lymphoma & related disorders

General

Staging-primary cutaneous


Resident / Fellow Advisory Board: Mario L. Marques-Piubelli, M.D.
Deputy Editor-in-Chief: Genevieve M. Crane, M.D., Ph.D.
Amrit P. Singh, M.D.
Aubrey J. Hough, M.D.

Topic Completed: 1 November 2021

Minor changes: 1 November 2021

Copyright: 2002-2021, PathologyOutlines.com, Inc.

PubMed Search: Staging [TI] cutaneous lymphoma

Amrit P. Singh, M.D.
Aubrey J. Hough, M.D.
Page views in 2020: 336
Page views in 2021 to date: 274
Cite this page: Singh AP, Hough AJ. Staging-primary cutaneous. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomanonBstagingprimarycutaneous.html. Accessed December 8th, 2021.
Definition / general
Essential features
  • Staging criteria for mycosis fungoides / Sézary syndrome (MF / SS) is different from non mycosis fungoides / Sézary syndrome lymphomas (including primary cutaneous B cell lymphomas [CBCL], other primary cutaneous T cell lymphomas [CTCL] and NK cell lymphomas) (Blood 2007;110:1713, Blood 2007;110:479)
ICD coding
  • ICD-10:
    • C82.6 - cutaneous follicle center lymphoma
    • C83.3 - diffuse large B cell lymphoma, unspecified site
    • C84.0 - mycosis fungoides, unspecified
    • C84.1 - Sézary disease
    • C84.A0 - cutaneous T cell lymphoma, unspecified
    • C86.0 - extranodal NK / T cell lymphoma, nasal type
    • C86.3 - subcutaneous panniculitis-like T cell lymphoma
    • C86.6 - primary cutaneous CD30 positive T cell proliferations
    • C88.4 - extranodal marginal zone B cell lymphoma of skin associated lymphoid tissue (SALT lymphoma)
Diagrams / tables

Images hosted on other servers:

Body surface area estimation

Body regions

Lymph node regions

TNMB classification of MF / SS

Lymph node grading

TNM classification of non-MF / SS

Primary tumor of skin (pT)
  • Mycosis fungoides and Sézary syndrome; this is the revised ISCL/EORTC staging of MF/SS (Blood 2007;110:1713)
    • pT1: involving < 10% of skin surface
      • pT1a: patch only
      • pT1b: plaque ± patch
    • pT2: involving > 10% of skin surface
      • pT2a: patch only
      • pT2b: plaque ± patch
    • pT3: 1 or more tumors > 1 cm in diameter
    • pT4: confluence of erythema covering > 80% of skin surface
  • Non mycosis fungoides / Sézary syndrome primary cutaneous lymphomas (Blood 2007;110:479)
    • pT1: solitary lesion
      • pT1a: < 5 cm diameter
      • pT1b: ≥ 5 cm diameter
    • pT2: multiple lesions limited to 1 body region or 2 contiguous body regions
      • pT2a: all disease encompassing a ≤ 15 cm diameter circular area
      • pT2b: all disease encompassing a > 15 and ≤ 30 cm diameter circular area
      • pT2c: all disease encompassing a > 30 cm diameter circular area
    • pT3: generalized skin involvement
      • pT3a: multiple lesions involving 2 noncontiguous body regions
      • pT3b: multiple lesions involving ≥ 3 body regions
Regional lymph nodes (pN)
  • Mycosis fungoides and Sézary syndrome (Blood 2007;110:1713)
    • pN0: no clinically abnormal peripheral lymph nodes; biopsy not required
    • pN1: clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0 - 2
      • pN1a: clone negative
      • pN1b: clone positive
    • pN2: clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3
      • pN2a: clone negative
      • pN2b: clone positive
    • pN3: clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3 - 4 or NCI LN4; clone positive or negative
    • pNx: clinically abnormal peripheral lymph nodes; no histologic confirmation
  • Non mycosis fungoides / Sézary syndrome primary cutaneous lymphomas (Blood 2007;110:479)
    • pN0: no clinically or pathologically abnormal lymph nodes
    • pN1: involvement of 1 peripheral lymph node region that drains an area of current or prior skin involvement
    • pN2: 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
    • pN3: involvement of central lymph nodes

Notes:
  • Mycosis fungoides and Sézary syndrome
    • 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
    • T cell clone is defined by PCR or Southern blot analysis of the T cell receptor gene
  • Non mycosis fungoides / Sézary syndrome primary cutaneous lymphomas
    • 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, para-aortic, iliac
Distant metastasis (pM)
  • Mycosis fungoides and Sézary syndrome (Blood 2007;110:1713)
    • pM0: no visceral organ involvement
    • pM1: visceral involvement with pathology confirmation
  • Non mycosis fungoides / Sézary syndrome primary cutaneous lymphomas (Blood 2007;110:479)
    • pM0: no evidence of extracutaneous non lymph node disease
    • pM1: extracutaneous non lymph node disease present
Blood involvement (pB)

Notes:
  • 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 1 of the following modified ISCL criteria along with a positive clonal rearrangement of the TCR may be used instead:
    • Expanded CD4+ or CD3+ cells with CD4/CD8 ratio of 10 or more
    • Expanded CD4+ cells with abnormal immunophenotype including loss of CD7 or CD26
  • T cell clone is defined by PCR or Southern blot analysis of the T cell receptor gene
Prefixes
Not standardized for primary cutaneous lymphomas, therefore not used currently
AJCC prognostic stage groups
Mycosis fungoides / Sézary syndrome (Amin: AJCC Cancer Staging Manual, 8th Edition, 2017)
Stage group IA:  T1   N0   M0   B0 - 1
Stage group IB:  T2   N0   M0   B0 - 1
Stage group IIA:  T1 - 2   N1 - 2   M0   B0 - 1
Stage group IIB:  T3   N0 - 2   M0   B0 - 1
Stage group III:  T4   N0 - 2   M0   B0 - 1
Stage group IIIA:  T4   N0 - 2   M0   B0
Stage group IIIB:  T4   N0 - 2   M0   B1
Stage group IVA1:  T1 - 4   N0 - 2   M0   B2
Stage group IVA2:  T1 - 4   N3   M0   B0 - 2
Stage group IVB:  T1 - 4   N0 - 3   M1   B0 - 2
Registry data collection variables
  • For MF/SS : peripheral blood involvement
Histologic grade (G)
Not done for primary cutaneous lymphomas
Histopathologic type
  • Cutaneous T cell lymphomas
    • Mycosis fungoides
      • Folliculotropic MF
      • Pagetoid reticulosis
      • Granulomatous slack skin
    • Sézary syndrome
    • Primary cutaneous anaplastic large cell lymphoma
    • Subcutaneous panniculitis-like T cell lymphoma
    • Extranodal NK/T cell lymphoma, nasal type
    • Primary cutaneous gamma delta T cell lymphoma
    • Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma
    • Primary cutaneous acral CD8+ T cell lymphoma
    • Primary cutaneous CD4+ small / medium T cell lymphoproliferative disorder
    • Primary cutaneous peripheral T cell lymphoma, NOS
  • Cutaneous B cell lymphomas
    • Primary cutaneous marginal zone lymphoma
    • Primary cutaneous follicle center lymphoma
    • Primary cutaneous diffuse large B cell lymphoma, leg type
Clinical images

Contributed by Henry K. Wong, M.D., Ph.D.
Mycosis fungoides, patch stage

Mycosis fungoides, patch stage

Mycosis fungoides, plaque stage

Mycosis fungoides, plaque stage

Mycosis fungoides, tumor stage Mycosis fungoides, tumor stage

Mycosis fungoides, tumor stage

Peripheral smear images

Images hosted on other servers:

Sézary cells, peripheral smear

Videos

Diagnosis and staging of cutaneous lymphoma

Board review style question #1

A 45 year old woman presents with new onset violaceous plaques and tumors on the lower leg and foot with rapid progression (see picture above). The lesions measure 3.2 cm and 4.9 cm in greatest dimension, respectively. No other abnormalities are found on physical exam. Biopsy of the lesions shows sheet of large, atypical cells that stain positively with CD20, MUM1 and BCL2. A diagnosis of diffuse large B cell lymphoma, leg type is made. What is the correct T stage of the disease?

  1. T1a
  2. T2a
  3. T3a
  4. T3b
Board review style answer #1
B. T2a. Multiple lesions of diffuse large B cell lymphoma, leg type are present on 2 contiguous regions of the body, hence, the T stage is T2a using the non mycosis fungoides / Sézary syndrome primary cutaneous lymphoma staging criteria.

Comment Here

Reference: Staging - primary cutaneous lymphoma
Board review style question #2
A 68 year old man presents with erythema of the entire back, chest, abdomen and bilateral extremities. On further inspection, there is a 1.8 cm raised ulcerated lesion present on the left upper thigh and multiple enlarged palpable lymph nodes in the inguinal region. Biopsy of the thigh lesion shows tagging of CD4 + T cells along the dermal epidermal junction and epidermotropism. The cells show loss of CD7 and CD26. What is the most likely diagnosis and correct T stage of the disease?

  1. Mycosis fungoides; stage T1a
  2. Mycosis fungoides; stage T2b
  3. Mycosis fungoides; stage T3
  4. Mycosis fungoides; stage T4
Board review style answer #2
D. Mycosis fungoides; stage T4. The erythema covers > 80% of body surface which is stage T4 according to the mycosis fungoides / Sézary syndrome primary cutaneous lymphoma staging criteria.

Comment Here

Reference: Staging - primary cutaneous lymphoma
Back to top
Image 01 Image 02