Primary cutaneous large B-cell lymphomas of the legs. A distinct type of cutaneous B-cell lymphoma with an intermediate prognosis. Dutch Cutaneous Lymphoma Working Group

Primary cutaneous B-cell lymphoma: A clinically homogeneous entity?

“…37 These have nonaggressive clinical behavior, with mostly localized disease, a good response to orthovolt radiotherapy, only a slight tendency to extracutaneous spread, and an excellent prognosis. In our experience 18 and in that of other groups (Berti E., unpublished data), a distinct subset of elderly patients characterized by lesion location on the legs, large-cell histology findings and intermediate prognosis 35 is not identifiable. Histologic features of primary CBCL include a characteristic polymorphism of the neoplastic cellular infiltrate, with cells resembling small centrocytes ("centrocyte-like cells"37), large anaplastic centrocytes and centroblasts of different subtypes, plasma and/or plasmacytoid cells, and immunoblasts.…”

“…It has a chronic course, is highly sensitive to radiotherapy, and has a good prognosis, with the exception of a recently defined subset (large-cell FCC lymphoma of the leg), which predominantly affects elderly patients and has an intermediate prognosis. 35 Primary cutaneous IC 36 is characterized clinically by one or several, often deeply seated nodules or tumors, almost without exception on arms or legs, and has an excellent prognosis. Histologic examination reveals lymphoplasmacytoid/plasma cells at the periphery of the nodular infiltrates, different from the dispersed distribution of the same cells in secondary cutaneous IC 36 and nodal IC.…”

EBV positivity in primary cutaneous large B-cell lymphoma with immunophenotypic features of leg type: An isolated incidence or something more significant?

“…In contrast to the group of PCFCLs, they more often disseminate to extracutaneous sites [10]. Histologically, these lymphomas show diffuse infiltrates of a monotonous population or sheets of centroblasts and immunoblasts [6,10] which often extend into the subcutaneous tissue. Mitotic figures are frequent, small B-cells are lacking and reactive T-cells are relatively few and often confined to perivascular areas.…”

“…However, it was noted from the first publications regarding these PCFCLs that patients presenting with skin tumors on the leg had a different clinical behavior [5]. Since these tumors of the leg were consistently composed of a majority of large B-cells, the term PCLBCL, leg type was used [6]. Recent studies have demonstrated that patients with PCLBCL, leg type differed from patients with PCFCL arising at other sites by an older age of onset, poorer prognosis, and the almost constant expression of bcl-2 protein [7].…”

BCA-1, A B-cell chemoattractant signal, is constantly expressed in cutaneous lymphoproliferative B-cell disorders

“…Subtle classification problems apart, in this paper we will refer to this prominent group of CBCL as ''low grade CBCL''. In addition, a third, much smaller subgroup with intermediate prognosis (the so-called large B-cell lymphoma of the leg) is identified in the EORTC classification [21,24]. The clinicopathological significance of this group of primary CBCL is currently debated.…”