Cutaneous Presentation of Follicular Lymphomas

Abstract

The description of primary cutaneous follicular lymphoma has raised interest in the differential diagnosis of this versus disseminated follicular lymphoma involving the skin. We report here on four cases of Stage IV follicular lymphoma, diagnosed in skin biopsy, in which cutaneous lesion was the most noticeable feature of clinical presentation. In all cases, the morphological features were superimposed over typical nodal follicular lymphoma. Apart from classic B-cell markers, they were characterized by CD10 and bcl6 positivity, markers of follicle germinal center cells; and bcl2 expression, with a corresponding t(14;18) translocation in three of three cases examined. In all four cases, bone marrow study and clinical staging revealed disease that had disseminated since diagnosis. Follow-up showed relapsing cutaneous and nodal disease in two cases. The only difference observed with a control group of 10 cases of primary cutaneous follicular lymphoma was the absence in this group of t(14; 18). Disseminated classical follicular lymphoma has to be considered in the differential diagnosis of follicular lymphoma presenting in the skin. This series of cases suggests that the presence of t(14;18) could imply the existence of disease that has disseminated beyond the skin and that cases harboring this translocation could be candidates for systemic polychemotherapy.

IgV and bcl6 somatic mutation analysis reveals the heterogeneity of cutaneous B-cell lymphoma, and indicates the presence of undisclosed local antigens

“…[1][2][3][4][5][6] Although the clinical behavior of primary cutaneous BCL is dramatically different from those diagnosed in lymph nodes, 1,2,5,7-9 the morphology is strikingly similar. Until now no clear immunophenotypical or molecular differences have been known, [10][11][12] except that t(14;18) occurs rarely in skin follicular lymphomas (FLs), while it is characteristic of nodal FLs. 10,[13][14][15] At the same time, it seems that primary cutaneous BCL constitutes a heterogeneous group encompassing tumors denominated as FL, marginal-zone BCL and diffuse large BCL, although the precise distinction of these different cutaneous BCLs is a matter of controversy.…”

“…Until now no clear immunophenotypical or molecular differences have been known, [10][11][12] except that t(14;18) occurs rarely in skin follicular lymphomas (FLs), while it is characteristic of nodal FLs. 10,[13][14][15] At the same time, it seems that primary cutaneous BCL constitutes a heterogeneous group encompassing tumors denominated as FL, marginal-zone BCL and diffuse large BCL, although the precise distinction of these different cutaneous BCLs is a matter of controversy. 1,2,5,7,[16][17][18] Underlying all the uncertainties about the knowledge and classification of cutaneous BCLs is the relative paucity of available information about distinctive molecular events, with the exception of the identification of local phenomena such as Borrelia burgdorferi infection, and the expression of skin-related adhesion molecules [19][20][21] in a subset of cutaneous BCLs.…”

Primary cutaneous lymphoma of scalp in a child: a case report

“…4 PCL extend outside the cranium first and hence present with scalp mass rather than any neurological signs. 5 Skin is the second most common site of extra nodal lymphomas 65% are T-Cell lymphomas and 25% are B-Cell lymphomas and 10% are rare variants. Only 15-25% of cutaneous lymphomas show extra cutaneous manifestations at the time of diagnosis and prognosis is good.…”

“…Only 15-25% of cutaneous lymphomas show extra cutaneous manifestations at the time of diagnosis and prognosis is good. 5 Cutaneous T-cell lymphomas constitute a heterogenous group of lymphoproliferative diseases characterized by a clonal expansion of mature post thymic T-cells that infiltrate the skin. In relation to prognosis, patients with primary cutaneous lymphoma generally have a greater probability of disease-free survival than patients with secondary cutaneous lymphoma, although the histological and immunohistochemical features of the secondary skin lesions are identical to those of the systemic disease.…”

Breast systemic follicular lymphoma in a man: a case report

“…PBL represents 1% of all non-Hodgkin lymphomas and 0.4% to 0.5% of all breast malignancies [4,5]. Its definition is quite narrow, being relative to patients who had no evidence of disease outside the breast or ipsilateral axillary lymph nodes [6]. Diffuse large B cell lymphoma (DLBCL) is the most common histotype of PBL, while mucosa-associated lymphoid tissue (MALT)-type lymphoma and follicular lymphoma (FL) are less frequent ones.…”