Primary testicular lymphoma
Cet article fait le point sur l'incidence du lymphome primitif du testicule, ses caractéristiques cliniques et ses facteurs pronostiques, passe en revue les études concernant l'efficacité de diverses stratégies thérapeutiques, puis analyse le rôle de la radiothérapie et l'intérêt des nouveaux traitements
Primary testicular lymphoma (PTL) is a rare, clinically aggressive form of extranodal lymphoma. The vast majority of cases are histologically diffuse large B-cell lymphoma, but rarer subtypes are clinically important and must be recognised. In this review we discuss the incidence, clinical presentation and prognostic factors of PTL and present a summary of the recent advances in our understanding of the pathophysiology which may account for the characteristic clinical features. Although outcomes for patients with PTL have historically been poor, significant gains have been made with the successive addition of radiotherapy, full-course anthracycline-based chemotherapy, rituximab and CNS-directed prophylaxis. We describe the larger retrospective series, prospective clinical trials and critically examine the role of radiotherapy. Although 3-weekly RCHOP with intrathecal methotrexate and locoregional RT is the current international standard of care, a substantial minority of patients progress, representing an unmet medical need. Finally, we discuss new treatment approaches and recent discoveries which may translate into improved outcomes for patients with PTL.
Blood , résumé, 2013