From the Guidelines
The treatment for a retroperitoneal lesion with diffuse infiltrate of atypical medium-sized lymphoid cells, as shown in the Core Needle Biopsy (CNB), should be R-CHOP21 × 6, as recommended by the ESMO clinical practice guidelines for diagnosis, treatment, and follow-up of diffuse large B-cell lymphoma 1.
Diagnosis and Treatment Approach
The pathology findings suggest a lymphoproliferative disorder, likely a non-Hodgkin lymphoma. To determine the exact lymphoma subtype, a complete hematologic workup including immunohistochemistry, flow cytometry, and molecular studies is necessary, as treatment varies significantly based on classification.
- The patient's age, International Prognostic Index (IPI), and feasibility of dose-intensified approaches should be considered when selecting a treatment strategy, as outlined in Table 3 of the ESMO guidelines 1.
- For patients ≤60 years with IPI low risk (aaIPI = 0) and no bulk, R-CHOP21 × 6 is the recommended treatment strategy 1.
- Consideration of CNS prophylaxis is crucial in patients at risk for CNS progression, regardless of age or IPI score 1.
Treatment Regimens
- R-CHOP21 × 6 is a standard treatment regimen for diffuse large B-cell lymphoma, consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone 1.
- Alternative regimens, such as R-ACVBP and sequential consolidation or R-CHOP21 × 6 + IF-RT on bulk, may be considered in selected patients 1.
- High-dose chemotherapy (HDCT) with autologous stem-cell transplantation (ASCT) may be considered in patients with intermediate-high risk or IPI high risk (aaIPI = 2, 3) 1.
Additional Considerations
- A PET/CT scan is essential for determining the disease extent and guiding treatment decisions.
- Involved-field radiation therapy may be added for localized disease.
- Consultation with a hematologist-oncologist is crucial for developing a personalized treatment plan based on lymphoma subtype, stage, and patient factors.
From the FDA Drug Label
The trial was planned to enroll 600 patients with 1:1 randomization. The randomization was stopped early for efficacy after 362 patients had been enrolled (181 in each arm) according to the first planned interim analysis result A total of 328 randomized patients, aged 6 months and older, were included in the efficacy analyses, of which one patient under 3 years of age received intravenous RITUXAN or non-U.S. -licensed rituximab in combination with LMB chemotherapy Demographic and disease characteristics of the randomized trial population are displayed in Table 13: Table 13: Demographics and Disease Characteristics of the Randomized Trial Population - ITT RITUXAN + LMB ChemotherapyN = 164LMB ChemotherapyN = 164 BSA= Body Surface Area, B-AL=B-Cell Acute Leukemia, DLBCL= Diffuse Large B-cell Lymphoma, NHL= Non-Hodgkin's Lymphoma , CNS= Central Nervous System Male82%84% Female18%17% Age (years) Median (range)8 (2, 17)7 (1, 17) Age group 6 months to less than 3 years0.6%4% 3 to less than 12 years71%71% 12 to less than 18 years29%25% BSA (m2) Median (range)1.0 (0.6, 2.3)0.97 (0.5, 2. 7) Therapeutic group Group B high-risk49%51% Group C140%40% Group C311%10% Disease Type B-AL37%34% Burkitt or Burkitt-like NHL55%56% DLBCL8%8% Bone marrow involvement45%45% CNS involvement27%27% Efficacy was established based on event-free survival (EFS), where an event was defined as occurrence of progressive disease, relapse, second malignancy, death from any cause, or non-response as evidenced by detection of viable cells in residue after the second CYVE course, whichever occurs first. The results are described in Table 14. Table 14: Overview of Efficacy Results (ITT population) AnalysisLMB(N = 164)R-LMB(N=164) CI = confidence interval; EFS = event-free survival; HR = hazard ratio; ITT = intent-to-treat
- Event-free survival rate was calculated based on 38 event † two-sided unstratified log-rank test, testing null hypothesis of equality of event-free survivorship in randomization arms R-LMB and LMB against alternative hypothesis "event-free survivorship in group R-LMB is higher than in LMB." The analysis was based on 53% of information where the p-value boundary was 0. 014. ‡ R-LMB Versus LMB EFS*28 events10 events Two-sided unstratified log-rank test p-value 0.0012† Adjusted Cox HR‡ 0.32 (90% CI: 0.17, 0.58) As of data cutoff date of 31 December 2017, there were 20 and 8 deaths reported in LMB arm and R-LMB arm, respectively, with an estimated overall survival (OS) HR of 0.36 (95% CI, 0.16 - 0. 81). No formal statistical test was conducted for overall survival and therefore the OS result is considered descriptive. 14. 3 Diffuse Large B-Cell NHL (DLBCL) The safety and effectiveness of RITUXAN were evaluated in three randomized, active-controlled, open-label, multicenter studies with a collective enrollment of 1854 patients Patients with previously untreated diffuse large B-cell NHL received RITUXAN in combination with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or other anthracycline-based chemotherapy regimens NHL Study 7 A total of 632 patients age greater than or equal to 60 years with DLBCL (including primary mediastinal B-cell lymphoma) were randomized in a 1:1 ratio to treatment with CHOP or R-CHOP. Patients received 6 or 8 cycles of CHOP, each cycle lasting 21 days All patients in the R-CHOP arm received 4 doses of RITUXAN 375 mg/m2 on Days –7 and –3 (prior to Cycle 1) and 48–72 hours prior to Cycles 3 and 5. Patients who received 8 cycles of CHOP also received RITUXAN prior to Cycle 7. The main outcome measure of the study was progression-free survival, defined as the time from randomization to the first of progression, relapse, or death Responding patients underwent a second randomization to receive RITUXAN or no further therapy. Among all enrolled patients, 62% had centrally confirmed DLBCL histology, 73% had Stage III–IV disease, 56% had IPI scores greater than or equal to 2, 86% had ECOG performance status of < 2, 57% had elevated LDH levels, and 30% had two or more extranodal disease sites involved Efficacy results are presented in Table 15. These results reflect a statistical approach which allows for an evaluation of RITUXAN administered in the induction setting that excludes any potential impact of RITUXAN given after the second randomization Analysis of results after the second randomization in NHL Study 7 demonstrates that for patients randomized to R-CHOP, additional RITUXAN exposure beyond induction was not associated with further improvements in progression-free survival or overall survival NHL Study 8 A total of 399 patients with DLBCL, age greater than or equal to 60 years, were randomized in a 1:1 ratio to receive CHOP or R-CHOP. All patients received up to eight 3-week cycles of CHOP induction; patients in the R-CHOP arm received RITUXAN 375 mg/m2 on Day 1 of each cycle The main outcome measure of the study was event-free survival, defined as the time from randomization to relapse, progression, change in therapy, or death from any cause Among all enrolled patients, 80% had Stage III or IV disease, 60% of patients had an age-adjusted IPI greater than or equal to 2, 80% had ECOG performance status scores less than 2, 66% had elevated LDH levels, and 52% had extranodal involvement in at least two sites. Efficacy results are presented in Table 15 NHL Study 9 A total of 823 patients with DLBCL, aged 18–60 years, were randomized in a 1:1 ratio to receive an anthracycline-containing chemotherapy regimen alone or in combination with RITUXAN. The main outcome measure of the study was time to treatment failure, defined as time from randomization to the earliest of progressive disease, failure to achieve a complete response, relapse, or death
The treatment for a retroperitoneal lesion with diffuse infiltrate of atypical medium-sized lymphoid cells, as shown in the Core Needle Biopsy (CNB), is Rituximab in combination with chemotherapy.
- The specific chemotherapy regimen may include CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or other anthracycline-based chemotherapy regimens.
- The dose of Rituximab is 375 mg/m2, administered on specific days in combination with chemotherapy. 2
From the Research
Treatment for Retroperitoneal Lesion with Diffuse Infiltrate of Atypical Medium-Sized Lymphoid Cells
The treatment for a retroperitoneal lesion with diffuse infiltrate of atypical medium-sized lymphoid cells, as shown in the Core Needle Biopsy (CNB), is typically chemotherapy.
- The standard treatment for diffuse large B-cell lymphoma (DLBCL) is rituximab plus a regimen of cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) 3.
- A case study of a patient with a cystic left retroperitoneal mass diagnosed with DLBCL via percutaneous biopsy, was started on chemotherapy treatment 4.
- For patients with relapsed or refractory DLBCL, salvage immunochemotherapy followed by high-dose chemotherapy and autologous stem cell transplantation is the standard-of-care second-line treatment 5.
- A clinical case of a patient with primary retroperitoneal DLBCL was treated with poly-chemotherapy using R-CHOP 6.
- Another case study of a patient with primary retroperitoneal DLBCL with sclerosis, which mimicked idiopathic retroperitoneal fibrosis, was diagnosed post-mortem 7.
Chemotherapy Regimens
- R-CHOP is a commonly used chemotherapy regimen for DLBCL, consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone 3, 6.
- Other chemotherapy regimens, such as R-EPOCH and R-HyperCVAD, may also be used for aggressive B-cell lymphomas 5.