• Myrna Candelaria, Luis F Oñate-Ocaña, Judith Corona-Herrera, Cristina Barrera-Carmona, Mayra Ponce-Martínez, Olga Gutiérrez-Hernández, Alejandro Avilés-Salas, and Bernardo Cacho-Díaz.
• Clinical Research Division, Instituto Nacional de Cancerología, Mexico City, Mexico.
• Rev Invest Clin. 2019 Jan 1; 71 (5): 349-358.

BackgroundThe outcome of patients with primary extranodal diffuse large B-cell lymphoma (PE-DLBCL) varies according to the primary site involved. Primary gastrointestinal, breast, bone, craniofacial, and testicular DLBCL are rare extranodal manifestations of DLBCL.ObjectiveThe objective of the study was to describe the clinical course of patients with PE-DLBCL disease in a referral cancer center.ResultsFrom 637 patients, 51 (8.77%) were considered as having PE-DLBCL (25 gastrointestinal, 12 craniofacial, 6 breast, 5 bone, and 3 with primary testicular DLBCL). Complete remission was higher in all PE-DLBCL sites (100% in testicular, 92.6% craniofacial, 83.3% breast, 80% bone, and 80% gastrointestinal) compared with 73.3% in nodal DLBCL. Although 2 cases with breast PE-DLBC relapsed, they achieved a complete response with chemotherapy. The overall survival at 5 years was 100%, 80%, 78%, 58%, 58%, and 62% for patients with primary breast, primary bone, gastrointestinal, primary craniofacial, primary testicular, and nodal DLBCL, respectively.ConclusionsPE-DLBCLs constitute rare, primary sites of lymphoproliferative disorders in most cases, with localized disease and good prognosis. They require a combined chemoimmunotherapy with radiotherapy in most cases to improve local and systemic disease.Copyright: © 2019 Permanyer.

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