T-cell prolymphocytic leukemia: Epidemiology and survival trends in the era of novel treatments.

Survival remains poor for T-cell prolymphocytic leukemia, though treatment in recent years, associated with access to novel therapies, and management at academic medical centers is associated with improved outcomes. There remains a critical need to improve the available treatment options for this population, and access to specialized academic medical centers, comprehensive supportive care, clinical trials, and early palliative care remains essential for T-PLL patients.

Effect of cumulative dose of brentuximab vedotin maintenance in relapsed/refractory classical Hodgkin lymphoma after autologous stem cell transplant: an analysis of real-world outcomes.

Sixteen cycles of Brentuximab vedotin (BV) after autologous stem cell transplant (ASCT) in high-risk relapsed/refractory classical Hodgkin lymphoma demonstrated an improved 2-year progression-free survival (PFS) over placebo. However, most patients are unable to complete all 16 cycles at full dose due to toxicity. This retrospective, multicenter study investigated the effect of cumulative maintenance BV dose on 2-year PFS. Data were collected from patients who received at least one cycle of BV maintenance after ASCT with one of the following high-risk features: primary refractory disease (PRD), extra-nodal disease (END), or relapse <12 months (RL<12) from the end of frontline therapy. Cohort 1 had patients with >75% of the planned total cumulative dose, cohort 2 with 51-75% of dose, and cohort 3 with ≤50% of dose. The primary outcome was 2-year PFS. A total of 118 patients were included. Fifty percent had PRD, 29% had RL<12, and 39% had END. Forty-four percent of patients had prior exposure to BV and 65% were in complete remission before ASCT. Only 14% of patients received the full planned BV dose. Sixty-one percent of patients discontinued maintenance early and majority of those (72%) were due to toxicity. The 2-year PFS for the entire population was 80.7%. The 2-year PFS was 89.2% for cohort 1 (n=39), 86.2% for cohort 2 (n=33), and 77.9% for cohort 3 (n=46) (P=0.70). These data are reassuring for patients who require dose reductions or discontinuation to manage toxicity.

Influence of racial and ethnic identity on overall survival in patients with chronic lymphocytic leukemia.

Overall survival for chronic lymphocytic leukemia (CLL) patients by race; propensity score matched by age, Charlson-Deyo comorbidity score, insurance, and income and education level of zip code of residence.

Impact of academic medical center access on outcomes in multiple myeloma.

Treatment at academic cancer centers (ACs) is associated with improved survival across hematologic malignancies, though the benefit in multiple myeloma (MM) has not been examined. This study aims to evaluate survival outcomes at Commission on Cancer accredited ACs compared to non-academic centers (NACs) for patients receiving MM-directed therapy. The National Cancer Database (NCDB) was used to identify demographics and overall survival (OS) of MM patients diagnosed from 2004 to 2017 and to compare outcomes by facility type. Survival analysis was repeated in a propensity score matched cohort, with NACs matched 1:1 to ACs by age, race, comorbidity score, insurance, year of diagnosis, distance traveled, and income. Of 163 375 MM patients, 44.5% were treated at ACs. Patients at ACs were more likely to receive MM-directed therapy compared to NACs (81% vs. 73%, p < .001). For patients receiving treatment, median OS at ACs was 71.3 months versus 41.2 months at NACs (p < .001). When adjusted for baseline demographics, patients treated at ACs had reduced mortality; hazard ratio (HR) 0.79 (95% CI 0.78-0.81, p < .001). The propensity score matched cohort maintained this survival benefit with a median OS of 59.9 months at ACs versus 37.0 months at NACs (p < .001), HR of 0.66 (95% CI 0.64-0.67, p < .001). ACs treated younger patients with fewer comorbidities and were more likely to treat racial minorities and patients with Medicaid or private insurance, and the uninsured. In this analysis, MM patients treated at ACs have significantly improved survival. While potentially related to access to specialized care, socioeconomic factors that drive facility selection may also contribute.

Survival Outcomes of Limited-Stage Diffuse Large B-Cell Lymphoma Treated With Radiation Therapy.

BACKGROUND: Patients with favorable risk limited-stage (LS) diffuse large b-cell lymphoma (DLBCL) have shown excellent outcomes without radiotherapy (RT). However, the role of RT for the remainder of LS-DLBCL patients is less well defined. We aimed to investigate whether the addition of RT provided an overall survival (OS) benefit in a real-world cohort of LS-DLBCL patients based on primary site at presentation. MATERIALS AND METHODS: Retrospective data from 39,745 patients with stage I and II DLBCL treated with front-line combination chemotherapy alone or followed by RT were identified using the National Cancer Database from 2004 to 2015. RESULTS: The addition of RT was associated with improved 5-year OS for all LS patients as compared to those treated with chemotherapy alone (85% vs. 80%, P < .001). RT was associated with improved 5-year OS in both the nodal and extranodal disease patients (nodal: 85% vs. 80%, P < .001; extranodal: 83% vs. 79%; P < .001). Extranodal sites with prolonged OS from the addition of RT include skin and soft tissue, head and neck, testicular, and thyroid sites (all P < .02). Breast, bone, lung and gastrointestinal extranodal primary sites had no OS benefit from the inclusion of RT. In multivariate analysis, the addition of RT was an independent factor for improved survival for all LS patients ([HR] 0.84, 95% [CI] 0.81-0.88; P < .001). CONCLUSION: Though there is no consensus on optimal treatment indications for RT in LS-DLBCL, these data suggest certain subgroups may have benefit when RT is added to front-line chemotherapy.

Frontline polatuzumab vedotin for diffuse large B-cell lymphoma: A survey of clinician impressions.

In the POLARIX trial, pola-R-CHP demonstrated improved progression-free survival (PFS) compared to R-CHOP in untreated intermediate- to high-risk DLBCL. We surveyed practicing clinicians regarding their interpretation of POLARIX, including impressions of efficacy, safety, and cost. Of 174 respondents, most from academic centers (82%) in the United States (57%), 70% stated they would not replace R-CHOP with pola-R-CHP due to insufficient PFS difference, lack of overall survival benefit, and excessive cost. Respondents not recommending pola-R-CHP expressed concerns about financial implications for both society and patients. We observed considerable heterogeneity in both study interpretation and plans for real-world implementation of pola-R-CHP.

Academic Centers Compared With Nonacademic Centers for Patients With International Prognostic Index Risk-stratified Diffuse Large B-cell Lymphoma: A Survival Outcomes Analysis.

INTRODUCTION: Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous disease associated with varying outcomes. The International Prognostic Index (IPI) has been the standard for the baseline prognostic assessment in these patients. The present study aimed to determine the effect of the treatment facility on the overall survival outcomes in patients with DLBCL stratified by IPI risk groups. MATERIALS AND METHODS: The National Cancer Database was used to identify patients with a diagnosis of DLBCL from 2004 to 2015. DLBCL was stratified by the IPI risk score from low- to high-risk disease, and the overall survival of those treated at academic centers was compared with that of those treated at nonacademic centers. RESULTS: Treatment at academic centers was associated with significantly improved overall survival for all patients with DLBCL (108.3 months) compared with those treated at nonacademic centers (74.5 months; P < .001). The median survival for patients with high-risk disease treated at academic centers (33.5 months) was more than twice that of high-risk patients treated at nonacademic centers (14.4 months; P < .001). The median survival for the other risk categories was similarly improved, although less pronounced in the lower IPI score groups. The long-term overall survival for all patients with DLBCL at academic centers was improved at 5 and 10 years (59% and 43% survival, respectively) compared with those treated at nonacademic centers (51% and 35% survival, respectively; P < .001). CONCLUSION: Patients with DLBCL treated at academic centers demonstrated improved survival compared with those treated at nonacademic centers, especially those with high-risk disease. Further investigations into the factors contributing to such disparities are required to help standardize care and improve outcomes.

Presentation of a Rare Malignancy: Leiomyosarcoma of the Prostate.

Prostatic leiomyosarcoma is an aggressive malignancy with a high risk of metastasis and a poor prognosis that poses unique diagnostic and treatment challenges.

Primary Thyroid Lymphoma: An Analysis of the National Cancer Database.

Introduction Primary thyroid lymphoma (PTL) is a rare malignancy, representing only 1% to 5% of thyroid malignancies and 2.5% to 7% of all extranodal lymphomas. Most cases of PTL are of B-cell origin, and 98% of all PTL cases are non-Hodgkin's lymphoma. Case series and case reports represent the majority of the available studies on PTL, with a paucity of large retrospective population studies available for this disease. This is the first National Cancer Database (NCDB) study completed on PTL and the only large retrospective study to examine the use of chemotherapy and immunotherapy in the treatment of this specific population. Methods The NCDB for non-Hodgkin's lymphoma was utilized to identify 3,466 patients diagnosed with PTL between 2004 and 2015. The database was used to examine demographic information including age, race, gender, histology, stage, and treatment modality. Bivariate Kaplan-Meier analysis with log-rank tests was used to analyze overall survival. Multivariate analysis was performed with Cox proportional hazards regression models to obtain hazard ratios to assess the association of patient characteristics and treatment methods with survival. Results The median all-cause survival for PTL was 11.6 years (95% confidence interval [CI]: 11.1 to 12.1 years). The majority of PTL patients were female (68%) and white (93%), with a mean age of 65.8 years. Histologically, 59.5% of cases were diffuse large B-cell lymphoma (DLBCL), 18.3% marginal zone lymphoma, 8% follicular lymphoma, and 1.9% Burkitt lymphoma. Regarding treatment, 40.6% received beam radiation, and 54% underwent surgical resection. Single-agent chemotherapy was used in only 3.5% of patients, where 60.7% received multiagent chemotherapy. Additionally, immunotherapy was used in 16.2% of patients. There was a significantly increased risk of mortality associated with increasing age, DLBCL histology, and higher disease stage. Multivariate analysis of treatment methods revealed that lobectomy (hazard ratio [HR]: 0.58, 95% CI: 0.47-0.73) and total or subtotal thyroidectomy (HR: 0.58, 95% CI: 0.47-0.71) had significantly improved survival rates over no surgical management (p < 0.001). Beam radiation (HR 0.67, 95% CI: 0.58-0.79) had a significant survival benefit over treatment regimens that did not include radiation therapy (p < 0.001). Multiagent (HR: 0.40, 95% CI: 0.33-0.49) and single-agent chemotherapy (HR: 0.43, 95% CI: 0.30-0.63) had significant improvement over treatment regimens that did not include chemotherapy (p < 0.001). Immunotherapy had a survival benefit (HR 0.87) although this was not found to be statistically significant (95% CI: 0.68-1.11). Other factors associated with decreased risk of mortality include treatment at academic medical centers (HR: 0.846) and integrated cancer centers (HR: 0.76) as compared to community centers (p < 0.05). Conclusion This is the largest study to date of PTL and the first to analyze the NCDB database. Patient characteristics, treatment modalities, and overall survival in PTL were examined to further characterize this rare disease. Beam radiation, chemotherapy, and surgical resection all reveal significant survival benefit, with multiagent chemotherapy having the greatest advantage.