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[Clinicopathologic characteristics and prognostic analysis of testicular diffuse large B-cell lymphoma]
1State Key Laboratory of Medical Genomics, Shanghai Institute of Hematology, Shanghai Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China.
Insights
Secondary testicular diffuse large B-cell lymphoma (DLBCL) has worse survival rates and lower remission rates than primary testicular DLBCL. Key prognostic factors include ECOG score, disease stage, LDH levels, and extra-nodal involvement.
Area of Science:
- Hematology and Oncology
- Molecular Biology
- Clinical Pathology
Context:
- Testicular diffuse large B-cell lymphoma (DLBCL) is a rare extranodal non-Hodgkin lymphoma.
- Understanding clinicopathologic features and prognostic factors is crucial for optimizing patient outcomes.
- Distinguishing between primary and secondary testicular DLBCL is important for treatment stratification.
Purpose:
- To analyze the clinicopathologic characteristics and prognosis of testicular DLBCL.
- To identify significant prognostic factors influencing survival and treatment response.
- To investigate the gene mutation profile in testicular DLBCL and its correlation with outcomes.
Summary:
- A retrospective analysis of 68 testicular DLBCL patients revealed that secondary testicular DLBCL is associated with advanced stage, higher LDH, poorer ECOG and IPI scores, and inferior 5-year progression-free survival (PFS) and overall survival (OS) rates compared to primary testicular DLBCL.
- Poor prognostic factors identified include ECOG score ≥2, Ann Arbor stages III-IV, elevated LDH levels, and multiple extra-nodal involvements.
- Commonly mutated genes in testicular DLBCL include PIM1, MYD88, CD79B, CREBBP, KMT2D, ATM, and BTG2. KMT2D mutations were more frequent in secondary DLBCL and correlated with poorer PFS.
Impact:
- Identifies secondary testicular DLBCL as a distinct entity with poorer prognosis, necessitating tailored treatment strategies.
- Provides a comprehensive list of clinical and molecular prognosticators for testicular DLBCL.
- Highlights the potential role of KMT2D mutations as a predictive biomarker for treatment response and survival in testicular DLBCL.
Abstract:
Objective: To analyze the clinicopathologic characteristics and prognosis of testicular diffuse large B-cell lymphoma (DLBCL) . Methods: A retrospective analysis was performed on 68 patients with testicular DLBCL admitted to Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine from October 2001 to April 2020. The gene mutation profile was evaluated by targeted sequencing (55 lymphoma-related genes) , and prognostic factors were analyzed. Results: A total of 68 patients were included, of whom 45 (66.2% ) had primary testicular DLBCL and 23 (33.8% ) had secondary testicular DLBCL. The proportion of secondary testicular DLBCL patients with Ann Arbor stage Ⅲ-Ⅳ (P<0.001) , elevated LDH (P<0.001) , ECOG score ≥ 2 points (P=0.005) , and IPI score 3-5 points (P<0.001) is higher than that of primary testicular DLBCL patients. Sixty-two (91% ) patients received rituximab in combination with cyclophosphamide, adriamycin, vincristine, and prednisone (R-CHOP) -based first-line regimen, whereas 54 cases (79% ) underwent orchiectomy prior to chemotherapy. Patients with secondary testicular DLBCL had a lower estimated 5-year progression-free survival (PFS) rate (16.5% vs 68.1% , P<0.001) and 5-year overall survival (OS) rate (63.4% vs 74.9% , P=0.008) than those with primary testicular DLBCL, and their complete remission rate (57% vs 91% , P=0.003) was also lower than that of primary testicular DLBCL. The ECOG scores of ≥2 (PFS: P=0.018; OS: P<0.001) , Ann Arbor stages Ⅲ-Ⅳ (PFS: P<0.001; OS: P=0.018) , increased LDH levels (PFS: P=0.015; OS: P=0.006) , and multiple extra-nodal involvements (PFS: P<0.001; OS: P=0.013) were poor prognostic factors in testicular DLBCL. Targeted sequencing data in 20 patients with testicular DLBCL showed that the mutation frequencies of ≥20% were PIM1 (12 cases, 60% ) , MYD88 (11 cases, 55% ) , CD79B (9 cases, 45% ) , CREBBP (5 cases, 25% ) , KMT2D (5 cases, 25% ) , ATM (4 cases, 20% ) , and BTG2 (4 cases, 20% ) . The frequency of mutations in KMT2D in patients with secondary testicular DLBCL was higher than that in patients with primary testicular DLBCL (66.7% vs 7.1% , P=0.014) and was associated with a lower 5-year PFS rate in patients with testicular DLBCL (P=0.019) . Conclusion: Patients with secondary testicular DLBCL had worse PFS and OS than those with primary testicular DLBCL. The ECOG scores of ≥2, Ann Arbor stages Ⅲ-Ⅳ, increased LDH levels, and multiple extra-nodal involvements were poor prognostic factors in testicular DLBCL. PIM1, MYD88, CD79B, CREBBP, KMT2D, ATM, and BTG2 were commonly mutated genes in testicular DLBCL, and the prognosis of patients with KMT2D mutations was poor.
