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Pathology of Primary Cutaneous Marginal Zone B-Cell Lymphoma

Dr Sampurna Roy MD                        

Dermatopathology Quiz Case 176

Diagnosis: Primary Cutaneous Marginal Zone B-cell Lymphoma 





Primary cutaneous marginal zone lymphoma (MZL) is a low-grade B-cell lymphoma that usually follows an indolent course. 

Extranodal marginal B-cell lymphoma of mucosa- associated lymphoid tissue (MALT) type is the preferred terminology (WHO).

Extranodal MZL has been described in a variety of extranodal locations, although skin was one of the last sites at which primary occurrence was documented.

This is probably due to the fact that identical lesions were already well recognised in skin under the heading of primary cutaneous immunocytoma, so called because of the lymphoplasmacytoid appearance of the constituent cells.

WHO criteria restricts the use of extranodal marginal zone lymphoma (MZL) to infiltrates composed mostly of small lymphocytes. 

This includes all cases previously reported as primary cutaneous immunocytoma and some examples of primary cutaneous follicle centre cell lymphoma where the neoplastic infiltrate is diffuse and prodominantly of small cell type.

The cases described as "cutaneous follicular lymphoid hyperplasia with plasma cell" are all considered examples of Extranodal marginal zone lymphoma.

According to some authors the term 'primary cutaneous immunocytoma' should be dropped since there is potential for confusion with what haematopathologists generally recognise as immunocytoma or lymphoplasmacytic lymphoma which is usually disseminated at presentation and associated with Waldenstrom's macroglobulinaemia.

Clinical features: Primary cutaneous marginal zone lymphoma usually occurs in young adults and adults.  Cases have been reported in children.

Elderly patients with cutaneous marginal zone lymphoma show prominent lymphoplasmacytoid appearance ( also called cutaneous immunocytoma).

The lesion usually presents as a red or reddish purple papules, nodules or tumours which may have a erythematous halo, or as infiltrated erythematous plaques.

Common sites are upper extremities, trunk, head and neck.

Lesions are often multiple and involvement of more than one skin area may occur.

Histopathological features:  Dermatopathology Quiz Case 176

Neoplastic infiltrates are usually nodular with a perivascular and periadnexal distribution, although some lesions may be predominantly diffuse. 

Under scanning magnification the tumour may show either top-heavy or bottom heavy infiltrate. Reactive germinal centres are present, the neoplastic infiltrate surrounding these structures in a marginal zone pattern or forming diffuse sheets between residual follicles.

Involvement of the subcutaneous fat is common. Epidermis is spared.

The malignant lymphocytes are of intermediate size with relatively abundant abundant pale staining or clear cytoplasm and round (monocytoid cells) or cleaved (centrocyte-like cells) nuclei. Scattered blast cells are always present.

Plasma cells are see in the majority of cases, and are arranged around the periphery of nodular lymphoid infiltrates or in a subepidermal location. Eosinophils may also frequently be present.

Lymphoepithelial lesions are rare unlike marginal zone lymphomas at other sites. Cases reported as immunocytoma represent a variation of this picture in which there is prominent lymphoplasmacytoid differentiation.



The neoplastic lymphocytes are CD20 positive except where they show prominent plasmacellular differentiation, in which case they are better identified with CD79a.

Almost all cases are bcl-2 positive.

The cells do not express CD5, CD10, cyclin-D1 or bcl6.

The neoplastic B-cells sometimes demonstrate CD43.

Kappa and lambda often demonstrate light chain restriction.

Spontaneous regression has been documented in rare cases and surgical excision appears to be effective in some instances, but radiotherapy is probably the treatment of choice.

A high percentage of patients suffer relapses which are often multiple but almost always confined to subcutaneous sites.

Prognosis is excellent with long-term survival.

Dissemination to other extranodal and nodal sites may occur in rare cases.

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Visit: Pathology of Intravascular Large B-cell Lymphoma

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Visit: Pathology of Primary Cutaneous Diffuse Large B-Cell Lymphoma


Further reading:

Detection of immunoglobulin light chain restriction in cutaneous B-cell lymphomas by ultrasensitive bright-field mRNA  in-situ hybridization.

Secondary cutaneous marginal zone B-cell lymphoma presenting as lipoatrophy in a patient with hepatitis C.

[Cutaneous B-cell lymphoma]

Cutaneous follicular lymphoid hyperplasia with monotypic plasma cells. A clinicopathologic study of 18 patients.

Primary cutaneous marginal zone lymphoma with sequential development of nodal marginal zone lymphoma in a patient with selective immunoglobulin A deficiency.

Primary cutaneous B-cell lymphomas: part I. Clinical features, diagnosis, and classification

Primary cutaneous B-cell lymphomas: part II. Therapy and future directions.

Primary cutaneous marginal zone B-cell lymphoma: An atypical case.

Primary cutaneous marginal zone lymphomas with plasmacytic differentiation show frequent IgG4 expression.

Primary cutaneous marginal zone lymphoma associated with juxta-articular fibrotic nodules in a teenager.

A comparative analysis of cutaneous marginal zone lymphoma and cutaneous chronic lymphocytic leukemia.

Eradication of Borrelia burgdorferi infection in primary marginal zone B-cell lymphoma of the skin.

Cutaneous lymphoid hyperplasia and cutaneous marginal zone lymphoma: comparison of morphologic and immunophenotypic features.

Are primary cutaneous immunocytoma and marginal zone lymphoma the same disease?

Infection by Borrelia burgdorferi and cutaneous B-cell lymphoma.




Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)







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