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ISCL/EORTC Revision to the Classification of Mycosis fungoides and Sézary Syndrome

Stage

T

N

M

Peripheral Blood Involvement

IA

T1

N0

M0

B0, B1

IB

T2

N0

M0

B0, B1

IIA

T1, T2

N1, N2

M0

B0, B1

IIB

T3

N0-2

M0

B0, B1

III

T4

N0-2

M0

B0, B1

IIIA

T4

N0-2

M0

B0

IIIB

T4

N0-2

M0

B1

IVA1

T1-4

N0-2

M0

B2

IVA2

T1-4

N3

M0

B0-2

IVB

T1-4

N0-3

M1

B0-2

From Olsen E et al: Revisions to the staging and classification of 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). Blood. 110(6):1713-22, 2007.
From Olsen E et al: Revisions to the staging and classification of 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). Blood. 110(6):1713-22, 2007.

Ann Arbor Classification and AJCC Stages/Prognostic Groups

I

Involvement of a single lymphatic site (i.e., nodal region, Waldeyer ring, thymus, or spleen) (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE) (rare in Hodgkin lymphoma).

II

Involvement of ≥ 2 lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE). The number of regions involved may be indicated by a subscript, as in, e.g., II3.

III

Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by the involvement of the spleen (IIIS) or both (IIIE,S). Splenic involvement is designated by the letter S.

IV

Diffuse or disseminated involvement of ≥ 1 extralymphatic organ, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s). Stage IV includes any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.

ISCL/EORTC Revision to the Classification of Mycosis fungoides and Sézary Syndrome

TNM

Definitions

Skin

 

T1

Limited patches,¹ papules, &/or plaques² covering < 10% of the skin surface; may further stratify into T1a (patch only) vs. T1b (plaque ± patch)

T2

Patches, papules, or plaques covering ≥ 10% of the skin surface; may further stratify into T2a (patch only) vs. T2b (plaque ± patch)

T3

1 or more tumors³ (≥ 1 cm diameter)

T4

Confluence of erythema covering ≥ 80% of body surface area

From Olsen E et al: Revisions to the staging and classification of 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). Blood. 110(6):1713-22, 2007.
¹"Patch" indicates any size skin lesion without significant elevation or induration. Presence/absence of hypo/hyperpigmentation, scale, crusting, &/or poikiloderma should be noted. 
²"Plaque" indicates any skin lesion that is elevated or indurated. Presence or absence of scale, crusting, &/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. 
³For skin, "tumor" indicates at least one 1 cm diameter solid or nodular lesion with evidence of depth &/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 is encouraged.

Node

 

N0

No clinically abnormal peripheral lymph nodes⁴; biopsy not required

N1

Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2

N1a

Clone negative5

N1b

Clone positive5

N2

Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3

N2a

Clone negative5

N2b

Clone positive5

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

Viscera

 

M0

No visceral organ involvement

M1

Visceral involvement (must have pathology confirmation,⁶ and organ involved should be specified)

Peripheral Blood Involvemen

 

B0

Absence of significant blood involvement: ≤ 5% of peripheral blood lymphocytes are atypical (Sézary) cells⁷

B0a

Clone negative⁵

B0b

Clone positive⁵

B1

Low blood tumor burden: > 5% of peripheral blood lymphocytes are atypical (Sézary) cells but does not meet the criterial of B2

B1a

Clone negative⁵

B1b

Clone positive⁵

B2

High blood tumor burden: ≥ 1,000/μL Sézary cells⁷ with positive clone⁵

From Olsen E et al: Revisions to the staging and classification of 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). Blood. 110(6):1713-22, 2007.
4For node, "abnormal peripheral lymph node(s)" indicates any palpable peripheral node that on physical examination is firm, irregular, clustered, fixed, or ≥ 1.5 cm in diameter. Node groups palpated 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. 
5A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor (TCR) gene. 
6For viscera, spleen and liver may be diagnosed by imaging criteria. 
7For blood, 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 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 ≥ 10; 2) expanded CD4(+) cells with abnormal immunophenotype including loss of CD7 or CD26.

Histopathologic Staging of Lymph Nodes in Mycosis fungoides and Sézary Syndrome

Updated ISCL/EORTC Classification

Dutch System

NCI-VA Classification

N1

Grade 1: Dermatopathic lymphadenopathy (DL)

LN0: No atypical lymphocytes

LN1: Occasional and isolated atypical lymphocytes (not arranged in clusters)

LN2: Many atypical lymphocytes or in 3-6 cell clusters

N2

Grade 2: DL; early involvement by MF (presence of cerebriform nuclei > 7.5 μm)

LN3: Aggregates of atypical lymphocytes; nodal architecture preserved

N3

Grade 3: Partial effacement of LN architecture; many atypical cerebriform mononuclear cells (CMCs)

LN4: Partial/complete effacement of nodal architecture by atypical lymphocytes or frankly neoplastic cells

Grade 4: Complete effacement

 

From Olsen E et al: Revisions to the staging and classification of 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). Blood. 110(6):1713-22, 2007.
From Olsen E et al: Revisions to the staging and classification of 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). Blood. 110(6):1713-22, 2007.

St. Jude Staging System

Stage

Definitions

I

A single tumor (extranodal) or single anatomic area (nodal), with the exclusion of mediastinum or abdomen

II

A single tumor (extranodal) with regional node involvement

≥ 2 nodal areas on the same side of the diaphragm

2 single (extranodal) tumors with or without regional node involvement on the same side of the diaphragm

A primary gastrointestinal tract tumor, usually in the ileocecal area, with or without involvement of associated mesenteric nodes only¹

III

2 single tumors (extranodal) on opposite sides of the diaphragm

≥ 2 nodal areas above and below the diaphragm

All primary intrathoracic tumors (mediastinal, pleural, thymic)

All extensive primary intraabdominal disease¹

All paraspinal or epidural tumors, regardless of other tumor site(s)

IV

Any of the above with initial central nervous system &/or bone marrow involvement²

From Murphy SB et al: Non-Hodgkin's lymphomas of childhood: An analysis of the histology, staging, and response to treatment of 338 cases at a single institution. J Clin Oncol. 7(2):186-93, 1989.
¹A distinction is made between apparently localized gastrointestinal tract lymphoma and more extensive intraabdominal disease because of their quite different patterns of survival after appropriate therapy. Stage II disease typically is limited to 1 segment of the gut ± the associated mesenteric nodes only and the primary tumor can be completely removed grossly by segmental excision. Stage III disease typically exhibits spread to paraaortic and retroperitoneal areas by implants and plaques in mesentery or peritoneum, or by direct infiltration of structures adjacent to the primary tumor. Ascites may be present, and complete resection of all gross tumor is not possible. 
²If the marrow involvement is present initially, the number of abnormal cells must be ≤ 25% in an otherwise normal marrow aspirate with a normal peripheral blood picture.

Χαρακτηριστικά Επέκτασης

  • Direct extension
  • Nodal involvement through lymphatic spread
    • Non-Hodgkin lymphoma typically spreads to noncontiguous lymph nodes, leading to widely disseminated pattern of nodal involvement
      • Non-Hodgkin lymphoma commonly spreads to extranodal sites
    • Hodgkin lymphoma typically spreads to contiguous lymph nodes, leading to pattern of nodal spread within same region
    • Hodgkin lymphoma rarely spreads to extranodal sites
  • Metastasis
  • Common sites include Spleen, Liver, Bone, Kidney

Κατηγοριοποίηση (Ιστολογικοί Τύποι)

Lymphoma broadly divided into 2 groups

  • Hodgkin lymphoma
  • Non-Hodgkin lymphoma

Primary malignant tumors (WHO classification)

  • B-cell neoplasm, NOS
    • Precursor B-cell neoplasms
      • Precursor B lymphoblastic leukemia
      • Precursor B lymphoblastic lymphoma
    • Peripheral (mature) B-cell neoplasms
      • Chronic lymphocytic leukemia (CLL)
      • Small lymphocytic lymphoma (SLL)
      • B-cell prolymphocytic leukemia
      • Lymphoplasmacytic lymphoma
      • Splenic marginal zone lymphoma
      • Hairy cell leukemia
      • Plasmacytoma/multiple myeloma
      • Solitary plasmacytoma of bone
      • Extraosseous plasmacytoma
      • Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
      • Nodal marginal zone B-cell lymphoma
      • Follicular lymphoma, NOS
      • Follicular lymphoma grade 1
      • Follicular lymphoma grade 2
      • Follicular lymphoma grade 3
      • Mantle cell lymphoma
      • Diffuse large B-cell lymphoma
      • Mediastinal (thymic) large cell lymphoma
      • Intravascular large B-cell lymphoma
      • Primary effusion lymphoma
      • Burkitt lymphoma/leukemia
      • B-cell proliferations of uncertain malignant potential
      • Lymphomatoid granulomatosis
      • Post-transplant lymphoproliferative disorder, pleomorphic
  • T-cell and NK-cell neoplasm, NOS
    • Precursor T-cell lymphoblastic leukemia
      • Precursor T lymphoblastic leukemia
      • Precursor T lymphoblastic lymphoma
      • Blastic NK-cell lymphoma
    • Peripheral (mature) T-cell neoplasms
      • T-cell prolymphocytic leukemia
      • T-cell large granular lymphocytic leukemia
      • Aggressive NK-cell leukemia
      • Adult T-cell leukemia/lymphoma
      • Extranodal NK-/T-cell lymphoma, nasal type
      • Enteropathy-type T-cell lymphoma
      • Hepatosplenic T-cell lymphoma
      • Subcutaneous panniculitis-like T-cell lymphoma
      • Mycosis fungoides
      • Sézary syndrome
      • Primary cutaneous anaplastic large cell lymphoma
      • Peripheral T-cell lymphoma, NOS
      • Angioimmunoblastic T-cell lymphoma
      • Anaplastic large cell lymphoma
      • Lymphomatoid papulosis
  • Hodgkin lymphoma, NOS
    • Nodular lymphocyte predominant Hodgkin lymphoma
    • Classical Hodgkin lymphoma
      • Nodular sclerosis classical Hodgkin lymphoma
      • Lymphocyte-rich classical Hodgkin lymphoma
      • Lymphocyte-depleted classical Hodgkin lymphoma