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- Primary testicular diffuse large B-cell lymphoma (PT-DLBCL), also termed testicular diffuse large B-cell lymphoma and diffuse large B-cell lymphoma of the testes, is a variant of the diffuse large B-cell lymphomas (DLBCL). DLBCL are a large and diverse group of B-cell malignancies with the great majority (-85%) being typed as diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS). PT-DLBCL is a variant of DLBCL, NOS that involves one or, in uncommon cases, both testicles. Other variants and subtypes of DLBCL may involve the testes by spreading to them from their primary sites of origin in other tissues. PT-DLBCL differs from these other DLBCL in that it begins in the testes and then may spread to other sites. The B-cells in PT-DLBCL are malignant lymphocytes that normally function in the humoral immunity component of the adaptive immune system by secreting antibodies that, for example, bind to and neutralize invasive pathogens. In ~75% of PT-DLBCL cases these malignant B-cells are termed "activated B-cells" to distinguish them from "germinal center B-cells". While DLBCL may involve either type of B-cell, the various forms and subtypes of the DLBCL that involve activated B-cells are more aggressive and may require different therapies than those involving germinal center B-cells. Lymphomas that begin in the testes, i.e. primary testicular lymphomas (PTL), are rare forms of lymphoma that represent 1-2% of all non-Hodgkins lymphomas and 5% of all testicular malignancies. PT-DLBCL is by far the most common form of PTL, accounting for >85% all PTL cases. Other forms of PTL include testicular mantle cell lymphoma, extranodal marginal zone B cell lymphoma, extranodal NK/T-cell lymphoma, nasal type, peripheral T-cell lymphoma, activin receptor-like kinase-1–negative anaplastic large cell lymphoma, and pediatric-type follicular lymphoma. Some European investigators have combined PT-DLBCL and most of the latter lymphomas into a lymphoma group termed "non-Hodgkin's primitive lymphoma of the testes". All forms of PTL except the pediatric-type follicular lymphoma are highly aggressive malignancies PT-DLBCL is no exception to this rule: it begins in a testis, often spreads to the contralateral (i.e. uninvolved) testis as well as to the central nervous system and other sites, and commonly recurs following its treatment. In consequence, the disease has had a 5-year cause-specific survival of only ~60%. Addition of the immunotherapeutic drug, rituximab, to standard chemotherapy protocols has somewhat improved the treatment of most cases of this disease. In addition, clinical trials are testing new drugs for preventing relapses in, as well as treating, PT-DLBCL. (en)
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| rdfs:comment
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- Primary testicular diffuse large B-cell lymphoma (PT-DLBCL), also termed testicular diffuse large B-cell lymphoma and diffuse large B-cell lymphoma of the testes, is a variant of the diffuse large B-cell lymphomas (DLBCL). DLBCL are a large and diverse group of B-cell malignancies with the great majority (-85%) being typed as diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS). PT-DLBCL is a variant of DLBCL, NOS that involves one or, in uncommon cases, both testicles. Other variants and subtypes of DLBCL may involve the testes by spreading to them from their primary sites of origin in other tissues. PT-DLBCL differs from these other DLBCL in that it begins in the testes and then may spread to other sites. (en)
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