Clinicopathological Features of Syncytial Variant Nodular Sclerosis Hodgkin lymphoma.

The syncytial variant of nodular sclerosis Hodgkin lymphoma (SV-NSHL) is uncommon and its clinicopathological features have not been well studied. In this study, we collected 142 cases of SV-NSHL. There were 76 (53.5%) males and 66 (46.5%) females with a median age of 28 years (range, 12-78); 59.9% were younger than 30 years of age. Patients usually presented with a mediastinal mass (97.6%) and often with bulky disease (35.7%) and advanced stages (3 or 4) (39.7%). Follow-up showed 61.1% of patients developed relapse after standard therapy. Morphologically, the neoplasms were composed of sheets or large clusters of pleomorphic neoplastic cells with a cohesive appearance that were frequently associated with necrosis. Neutrophils and eosinophils were frequent in the background while small lymphocytes were decreased. Immunohistochemical analysis showed the following markers frequently to be positive: CD30 (100%), MUM1/IRF4 (96%), PAX5 (95%), PD-L1 (91%), CD15 (80%). CD45 was negative in all cases. Epstein-Barr virus-encoded small RNA (EBER) was detected in 19% of cases. In this cohort, age over 30 years (P = .0430), presence of B-type symptoms (P = .0394), elevated serum LDH level (P = .0004), and disease relapse (P = .0108) were associated with a poorer overall survival. In contrast, patients with EBER positive neoplasms had a better survival (P = .0418). Compared with a control group of non-SV-NSHL patients, patients with SV-NSHL were associated with a poorer overall survival (P = .011). These data suggest that SV morphology is associated with a poorer prognosis in patients treated with traditional standard-of-care therapy for classic Hodgkin lymphoma.