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Central nervous system (CNS) relapse affects 5% of diffuse large B-cell lymphoma (DLBCL) patients and portends a poor prognosis. Prophylactic intravenous high-dose methotrexate (HD-MTX) is frequently employed to reduce this risk, but there is limited evidence supporting this practice. We conducted a multicenter retrospective study to determine the CNS relapse risk with HD-MTX in DLBCL patients aged 18-70 years treated in Alberta, Canada between 2012 and 2019. Provincial guidelines recommended HD-MTX for patients at high-risk of CNS relapse based upon CNS-IPI score, double-hit lymphoma, or testicular involvement. Among 906 patients with median follow-up 35.3 months (range 0.29-105.7), CNS relapse occurred in 1.9% with CNS-IPI 0-1, 4.9% with CNS-IPI 2-3, and 12.2% with CNS-IPI 4-6 (p < .001). HD-MTX was administered to 115/326 (35.3%) high-risk patients, of whom 96 (83.5%) had CNS-IPI score 4-6, 45 (39.1%) had double-hit lymphoma, and four (3.5%) had testicular lymphoma. The median number of HD-MTX doses was two (range 1-3). Central nervous system relapse risk was similar with versus without HD-MTX (11.2% vs. 12.2%, p = .82) and comparable to previous reports of high-risk patients who did not receive CNS prophylaxis (10-12%). In multivariate and propensity score analyses, HD-MTX demonstrated no association with CNS relapse, progression-free survival, or overall survival. This study did not demonstrate a benefit of prophylactic HD-MTX in this high-risk patient population. Further study is required to determine the optimal strategy to prevent CNS relapse in DLBCL.

A 27-year-old Chinese male presented with multiple myeloma. Over his 18-month course he manifested a number of unusual features of his disease including his young age, marked organomegaly, a testicular plasmacytoma, multiple intracranial extraskeletal plasmacytomas and meningeal involvement, and peripheral blood plasmacytosis. The case is described and recent literature on these rare manifestations is reviewed.

The Ann Arbor staging was originally developed in 1971 for Hodgkin lymphoma at a time when radiation was the primary treatment [1]. It was subsequently adopted for use in non-Hodgkin lymphoma (NHL). Although the Ann Arbor staging continues to be valuable in nodal NHL, its application in extranodal NHL has limitations [2,3]. Proper staging and accurate prognosis of diffused large B-cell lymphoma (DLBCL) with isolated bilateral testicular involvement are unclear. Here we show that even lymphoma experts do not have a consensus on these issues with 60.7% choosing Stage IV and the remaining favoring either Stage I E or II E . To determine the prognosis of DLBCL patients with isolated bilateral testicular involvement, we performed disease-specific survival analyses using data from the Surveillance Epidemiology and End Results Program (SEER). The survival was not significantly different from age-matched Stage II unilateral disease (P = 0.549) and was better when compared with Stage IV unilateral disease (P = 0.034). Our study suggests that a limited stage designation is more appropriate due to a relatively better prognosis. To provide uniform staging for clinical trial and prognostic purposes, development of site- and histology-specific modified staging for primary extranodal lymphoma involving paired organs may be warranted.

We report an 8-year-old boy with Ph-positive chronic myelogenous leukemia (CML) who showed a testicular tumor as the first manifestation of blastic crisis. Pathological examination of the excised testis revealed marked infiltration of immature cells, whereas bone marrow remained in the chronic phase. Immunohistochemical and molecular examinations disclosed the immature cells to have B-lymphocyte surface determinants and immunoglobulin JH rearrangement. DNA obtained from the testis and peripheral blood cells showed similarly rearranged bcr configurations, indicating that infiltrating cells in the testis originated from the CML clone. Bone marrow showed B-lymphoid blastic crisis 4 months after testicular involvement.