Superior vena cava syndrome and poor performance status at presentation affect survival in mediastinal T-lymphoblastic lymphoma—a single institute experience from India

Abstract

Analysis of mediastinal T-lymphoblastic lymphomas (T-LBL) with T-acute lymphoblastic leukemia has precluded identification of prognostic factors. Newly diagnosed T-LBL patients presenting with mediastinal mass and <20% bone marrow involvement were analyzed for clinical features and outcome. In 55 patients with median age 18 years, 39 (71%) had "B" symptoms, 46 (83.6%) had bulky mediastinal mass and two thirds of patients (n = 36) had superior vena cava syndrome/superior mediastinal syndrome (SVCS/SMS). With acute lymphoblastic leukemia (ALL)-like protocols in majority, complete remission was achieved in 58.2% patients. Estimated 5-year overall survival (OS) and event-free survival (EFS) were 59.8 and 51.6% (median follow-up-35 months). On multivariate analysis, poor Eastern Cooperative Oncology Group (ECOG) performance status (PS > 2; n = 14) affected OS (p = 0.007), while Poor ECOG PS and SVCS/SMS affected EFS (p = 0.008 and p = 0.035, respectively). Combination of baseline-poor PS and presence of SVCS/SMS predicted poor EFS in a prognostic model (HR 6.20; p = 0.002). This is the first outcome study from Asia which has been able to predict baseline ECOG PS and presence of SVCS/SMS as prognostic factors affecting EFS.

Allogeneic hematopoietic stem cell transplantation should be in preference to conventional chemotherapy as post-remission treatment for adults with lymphoblastic lymphoma

“…Despite that the Coleman system has been widely accepted, most further studies failed to identified these prognostic factors. Our risk factor analysis indicated poor ECOG-PS at diagnosis was an independent risk factor for both OS and PFS, which was in accordance with previous conclusions [ 2 , 15 ]. One possible explanation was that higher tumor burden resulted in poorer performance status.…”

Pediatric superior vena cava syndrome: An evidence‐based systematic review of the literature

“…Two patients experienced cardiac arrest during diagnostic procedures, one of whom died. In contrast, Tilak et al . reported a cohort where 36/55 (65.5%) patients with T‐cell lymphoma presented with SVCS, 71% had a pleural effusion and 52% had a pericardial effusion.…”

“…Surgical intervention via open‐heart surgery was needed in 27% of cases (n = 38/142), with a higher rate of complications compared to catheterization (n = 5, 13%). In relation to oncologic treatment, it is important to note that acute deterioration and death directly attributable to SVCS, with an inability to initiate any therapy, occurred in five patients (13%), which is slightly higher than the mortality rate of 9% found in the literature . Table summarizes the details of all the different treatment modalities used in the management of SVCS in children, including the duration and presence of complications where applicable.…”

“…There are a small number of retrospective cohort studies but most are not generalizable since they include both adults and children in the same cohort 4,6,9,10,[12][13][14][15][16][17] or they focus on a specific subgroup of SVCS patients, 2,3,5,11,18,19 as outlined in Supplementary Table S5. One of the studies, conducted by Tzifa et al, was a single-center retrospective cohort study that examined the characteristics of cardiac versus noncardiac SVCS.…”

“…and death directly attributable to SVCS, with an inability to initiate any therapy, occurred in five patients (13%), which is slightly higher than the mortality rate of 9% found in the literature. 12 Table 1 summarizes the details of all the different treatment modalities used in the management of SVCS in children, including the duration and presence of complications where applicable.…”