Primary marginal zone lymphoma of the thymus.

Primary low-grade B-cell lymphomas of the thymus are rare, with only 7 reported cases in the literature. We describe 3 cases of primary low-grade thymic lymphoma. All had histologic features of extranodal marginal zone lymphoma and were composed predominantly of small lymphocytes with variable components of monocytoid cells and plasma cells. Overt transformation to large cell lymphoma occurred in 1 case. The neoplastic cells were immunoreactive for the B-cell marker CD20 and were positive for bcl-2 in 2 cases. Two of 3 patients had a long-standing history of autoimmune disease. Based on these findings and those of previously reported cases, marginal zone lymphoma is the predominant type of low-grade thymic B-cell lymphoma. These tumors seem to be more common in patients with autoimmune disorders, and as observed with marginal zone lymphoma arising at other anatomic sites, they may undergo transformation to a higher grade lymphoma.