Sarcoma: Disease Overview and Treatment Updates.

During JADPRO Live Virtual 2020, Cissimol Joseph, APRN, AOCNP®, and Prachee Singh, PA-C, MS, highlighted the clinical features and diagnostic workup of soft-tissue sarcoma and presented on a multidisciplinary approach to treatment planning.

Prognostic impact of corticosteroids on efficacy of chimeric antigen receptor T-cell therapy in large B-cell lymphoma.

Corticosteroids are commonly used for the management of severe toxicities associated with chimeric antigen receptor (CAR) T-cell therapy. However, it remains unclear whether their dose, duration, and timing may affect clinical efficacy. Here, we determined the impact of corticosteroids on clinical outcomes in patients with relapsed or refractory large B-cell lymphoma treated with standard of care anti-CD19 CAR T-cell therapy. Among 100 patients evaluated, 60 (60%) received corticosteroids for management of CAR T-cell therapy-associated toxicities. The median cumulative dexamethasone-equivalent dose was 186 mg (range, 8-1803) and the median duration of corticosteroid treatment was 9 days (range, 1-30). Corticosteroid treatment was started between days 0 and 7 in 45 (75%) patients and beyond day 7 in 15 (25%). After a median follow-up of 10 months (95% confidence interval, 8-12 months), use of higher cumulative dose of corticosteroids was associated with significantly shorter progression-free survival. More importantly, higher cumulative dose of corticosteroids, and prolonged and early use after CAR T-cell infusion were associated with significantly shorter overall survival. These results suggest that corticosteroids should be used at the lowest dose and for the shortest duration and their initiation should be delayed whenever clinically feasible while managing CAR T-cell therapy-associated toxicities.

Outcome of relapsed and refractory nodular lymphocyte-predominant Hodgkin lymphoma: a North American analysis.

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare entity, with limited data on the outcome in the relapsed/refractory setting. We evaluated the outcome of all patients diagnosed between 04/1979 and 01/2019 with relapsed or progressive NLPHL after initial active therapy at two institutions, refractory disease being defined as lack of response to treatment and/or relapse within three months of treatment. NLPHL patients with histological evidence of transformation at time of first relapse or progression were excluded. In total, 69 patients with recurrent NLPHL were included in the study. After a median follow-up after initial diagnosis of 14 years (range, 0·5-46 years), median progression-free survival after front-line treatment (PFS-1) was four years. Second-line therapy included chemotherapy in 28 (41%) patients, biological therapy (rituximab, lenalidomide or brentuximab vedotin) in 14 (20%), high-dose chemotherapy followed by autologous stem cell transplant in 14 (20%) and radiation therapy (RT) alone in 10 (15%). The five-year PFS after second-line therapy (PFS-2) was 68% [95% confidence interval (CI), 54-79%] but the five-year overall survival (OS) after second-line therapy (OS-2) remained excellent, at 94% (95% CI, 85-99%). Due to excellent outcome in case of recurrence, studies aimed at characterizing its biology to guide therapy de-escalation are needed.

Clinical and radiologic correlates of neurotoxicity after axicabtagene ciloleucel in large B-cell lymphoma.

Neurotoxicity or immune effector cell-associated neurotoxicity syndrome (ICANS) is the second most common acute toxicity after chimeric antigen receptor (CAR) T-cell therapy. However, there are limited data on the clinical and radiologic correlates of ICANS. We conducted a cohort analysis of 100 consecutive patients with relapsed or refractory large B-cell lymphoma (LBCL) treated with standard of care axicabtagene ciloleucel (axi-cel). ICANS was graded according to an objective grading system. Neuroimaging studies and electroencephalograms (EEGs) were reviewed by an expert neuroradiologist and neurologist. Of 100 patients included in the study, 68 (68%) developed ICANS of any grade and 41 (41%) had grade ≥3. Median time to ICANS onset was 5 days, and median duration was 6 days. ICANS grade ≥3 was associated with high peak ferritin (P = .03) and C-reactive protein (P = .001) levels and a low peak monocyte count (P = .001) within the 30 days after axi-cel infusion. Magnetic resonance imaging was performed in 38 patients with ICANS and revealed 4 imaging patterns with features of encephalitis (n = 7), stroke (n = 3), leptomeningeal disease (n = 2), and posterior reversible encephalopathy syndrome (n = 2). Abnormalities noted on EEG included diffuse slowing (n = 49), epileptiform discharges (n = 6), and nonconvulsive status epilepticus (n = 8). Although reversible, grade ≥3 ICANS was associated with significantly shorter progression-free (P = .02) and overall survival (progression being the most common cause of death; P = .001). Our results suggest that imaging and EEG abnormalities are common in patients with ICANS, and high-grade ICANS is associated with worse outcome after CAR T-cell therapy in LBCL patients.

Anaplastic Large Cell Lymphoma of the Breast Arising in a Burn Cicatrix.

Anaplastic large cell lymphoma (ALCL) is a T cell lymphoma characterized by large anaplastic cells with diffuse and uniform CD30 reactivity. Here, we report a patient with no prior implant history who developed anaplastic lymphoma kinase-1-negative ALCL within a burn cicatrix on a breast. We hypothesize that the chronic inflammation caused by burn injury and the patient's history of immunosuppression secondary to organ transplantation may have contributed to development of ALCL in this patient. This report supports the essential role of chronic inflammation in the development of ALCL in the breast. Level of Evidence: 5.

Tools to expedite the development of treatment plans.

Quality improvement strategies can be used to modify existing health care processes to reduce patient wait times. We undertook a quality improvement project to reduce the time between new patients' initial visits and the finalization of their treatment plans. Initiation of treatment of new patients at the MD Anderson Sarcoma Medical Oncology Clinic can take up to 2 weeks from their initial consultation. Treatment delays result in increased costs and anxiety for the patient, adversely affecting the quality of care provided. We performed detailed process mapping and a cause-and-effect analysis to identify and prioritize opportunities for improvement. Process improvements addressed 2 key causes of delay to develop a finalized treatment plan: (1) insufficient data for decision making at the time of new patient visit and (2) delays in obtaining diagnostic imaging. After implementing our process improvements, the median time to develop a treatment plan decreased by 89% from 70.5 to 7.6 hours. Our process changes involved minimal additional work and had the secondary outcome of resulting in time savings for the clinic team.

Air travel after biopsy-related pneumothorax: is it safe to fly?

PURPOSE: To evaluate the safety of air travel after percutaneous transthoracic needle biopsy (PTNB). MATERIALS AND METHODS: The study population included 179 patients who underwent 183 PTNBs followed by air travel within 14 days of the procedure. Patients were contacted after their flight and asked to complete a brief telephone survey that assessed for the development of respiratory symptoms during air travel. RESULTS: No patient reported experiencing an in-flight medical event that required emergent, in-flight medical attention or flight diversion. Postbiopsy pneumothorax developed in 65 patients. Of patients with postbiopsy pneumothorax, including patients with radiographic evidence of residual pneumothorax, 50 (77%) traveled within 4 days of the final postbiopsy chest radiograph. Worsening of existing respiratory symptoms or the development of new respiratory symptoms during or after the flight was reported in 14 of 183 patients (8%). CONCLUSIONS: This study shows that air travel after biopsy-related pneumothorax can occur safely before radiographic resolution of pneumothorax and as soon as 24 hours after PTNB.