Cutaneous Lymphoma

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August 2009

Skin Tumours ; Role of immunohistochemistry in Dermatopathology ; Cutaneous Pseudolymphoma; Lymphocytic Interstitial Pneumonia / Follicular Bronchiolitis; Pulmonary Lymphoproliferative Disease; Lymphomatoid Granulomatosis; B-cell non-Hodgkin’s MALT lymphomas; Post-Transplant Lymphoproliferative Disease ; Cardiac lymphoma; Gastric Lymphoma ;Lymphoma of the small intestine ;

What to include in the path report in a case of cutaneous lymphoproliferative lesion? CLICK HERE (power-point presentation)

The diagnosis of cutaneous lymphoid tumours of skin is one of the most difficult areas of dermatotopathology. The widely used classifications of lymphoma and cutaneous lymphoid tumours are the Revised European-American classification of Lymphoid neoplasms (the R.E.A.L classification) and the European Organisation for Research and Treatment of Cancer (E.O.R.T.C) . The R.E.A.L classification is based on a combination of histological, clinical (including site of presentation), biological, immunohistochemical and cytogenetic features.
The EORTC classification includes only primary cutaneous lymphomas and puts more emphasis on clinical features.
In Asia primary cutaneous T-cell lymphomas are more common ( 60%) compared to primary cutaneous B-cell lymphomas ( about 20%).

Classification of Cutaneous T-cell Lymphoma:

T- cell	EORTC classification	REAL classification
T cell	Mycosis Fungoides CLICK	Mycoses fungoides
T cell	MF associated with follicular mucinosis CLICK	x
T cell	Pagetoid reticulosis CLICK	x
T cell	Granulomatous slack skin (provisional) CLICK	x
T cell	Sezary syndrome CLICK	Sezary syndrome
T cell	Lymphomatoid papulosis CLICK	x
T cell	CD30 (+) large T-cell lymphoma CLICK anaplastic pleomorphic immunoblastic	Anaplastic large cell lymphoma Peripheral T-cell lymphoma Peripheral T-cell lymphoma (unspecified)
T cell	CD30 (-) large T-cell lymphoma CLICK pleomorphic large cell immunoblastic	Peripheral T-cell lymphoma ( unspecified )
T cell	Pleomorphic, small/medium sized cell Provisional CLICK	Peripheral T-cell lymphoma ( unspecified )
T cell	Subcutaneous panniculitis-like T-cell lymphoma Provisional CLICK	Subcutaneous panniculitis like T-cell lymphoma

Classification of Cutaneous B- cell Lymphoma:

B- cell	EORTC classification	REAL classification
B cell	Follicle center cell lymphoma (mainly on head and trunk) Indolent CLICK	Follicle centre cell lymphoma i)predominantly small cell ii) mixed small and large cell iii) predominantly large cell Diffuse large B- cell lymphoma
B cell	Immunocytoma/marginal zone B- cell lymphoma Indolent CLICK	Extranodal marginal zone B- cell lymphoma (MALT type)
B cell	Plasmacytoma Provisional	Plasmacytoma
B cell	Large B- cell lymphoma of the leg Intermediate CLICK	Diffuse large B- cell lymphoma
B cell	Intravascular large B- cell lymphoma Provisional CLICK	x

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Further Reading:

-Fink-Puches R et al. Primary cutaneous lymphomas: applicability of current classification schemes. (European Organization for research and treatment of Cancer, World Health Organization) based on clinicopathologic features observed in a large group of patients. Blood 2002; Vol 99. No3 : 800-805

-Williemze R ,Kerl H et al . EORTC classification for primary cutaneous lymphoma: a proposal from the cutaneous lymphoma study group of the European organization for Research and Treatment of cancer. Blood 1997;90 (1): 354-371.

- Harris NL et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood 1994;84;1361-1392

- Jaffe ES et al. Cutaneous lymphomas: a proposal for a unified approach to classification using the REAL/WHO Classification. Ann Oncol 2000;(Supp1);17-21

- Sander CA et al. The revised European - American Classification of Lymphoid neoplasms (REAL): a new perspective for the classification of cutaneous lymphomas. J Cutan Pathol 1997 Jul; (6): 329-41

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