Background: Primary testicular lymphoma (PTL) is a rare subtype of non-Hodgkin’s lymphoma (NHL), most commonly presenting as diffuse large B-cell lymphoma (DLBCL). It primarily affects older men and has a tendency to relapse in the central nervous system (CNS), contralateral testis, and lungs. Although rituximab has improved survival outcomes, its limited CNS penetration is a clinical challenge.
Case Report: We report the case of a 39-year-old man diagnosed with primary testicular DLBCL (PT-DLBCL) (triple expresser). He received six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin (hydroxydaunorubicin), vincristine (oncovin), and prednisone) combined with high-dose methotrexate (HD-MTX) and contralateral testicular radiation. After 33 months of disease-free survival, he experienced a CNS relapse. He was subsequently treated with the MAR (methotrexate, cytarabine, and rituximab) regimen, consisting of HD-MTX, cytarabine (Ara-C), and rituximab. Following whole-brain radiotherapy and rituximab maintenance therapy, he achieved complete response and was transitioned to maintenance therapy with acalabrutinib.
Conclusion: This case highlights the successful use of the MAR regimen and acalabrutinib maintenance in a patient with CNS-relapse PT-DLBCL. Further studies are needed to develop standardized treatment protocols for such high-risk cases.