Treatment with dose-adjusted EPOCH (etoposide, doxorubicin, cyclophosphamide, vincristine, prednisone) chemotherapy and rituximab (DA-EPOCH-R) has become the standard of care for primary mediastinal B-cell lymphoma (PMBCL) at many institutions despite limited data in the multi-centre setting. We report a large, multi-centre retrospective analysis of children and adults with PMBCL treated with DA-EPOCH-R to characterize outcomes and evaluate prognostic factors. We assessed 156 patients with PMBCL treated with DA-EPOCH-R across 24 academic centres, including 38 children and 118 adults. All patients received at least one cycle of DA-EPOCH-R. Radiation therapy was administered in 14·9% of patients. With median follow-up of 22·6 months, the estimated 3-year event-free survival (EFS) was 85·9% [95% confidence interval (CI) 80·3-91·5] and overall survival was 95·4% (95% CI 91·8-99·0). Outcomes were not statistically different between paediatric and adult patients. Thrombotic complications were reported in 28·2% of patients and were more common in paediatric patients (45·9% vs. 22·9%, P = 0·011). Seventy-five per cent of patients had a negative fluorodeoxyglucose positron emission tomography (FDG-PET) scan at the completion of DA-EPOCH-R, defined as Deauville score 1-3. Negative FDG-PET at end-of-therapy was associated with improved EFS (95·4% vs. 54·9%, P < 0·001). Our data support the use of DA-EPOCH-R for the treatment of PMBCL in children and adults. Patients with a positive end-of-therapy FDG-PET scan have an inferior outcome.
Increasing cure rates for childhood cancers have resulted in a population of adult childhood cancer survivors (CCS) that are at risk for late effects of cancer-directed therapy. Our objective was to identify facilitators and barriers to primary care physicians (PCPs) providing late effects screening and evaluate information tools PCPs perceive as useful. We analyzed surveys from 351 practicing internal medicine and family practice physicians nationwide. A minority of PCPs perceived that their medical training was adequate to recognize late effects of chemotherapy (27.6%), cancer surgery (36.6%), and radiation therapy (38.1%). Most PCPs (93%) had never used Children’s Oncology Group guidelines, but 86% would follow their recommendations. Most (84–86%) PCPs stated that they had never received a cancer treatment summary or survivorship care plan but (>90%) thought these documents would be useful. PCPs have a low level of awareness and receive inadequate training to recognize late effects. Overall, PCPs infrequently utilize guidelines, cancer treatment summaries, and survivorship care plans, although they perceive such tools as useful. We have identified gaps to address when providing care for CCS in routine general medical practice.
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