Pericardial involvement at diagnosis in pediatric Hodgkin lymphoma patients

BACKGROUND: Because most cases are clinically silent, the incidence, clinical course, and outcome of pericardial involvement in Hodgkin lymphoma are unknown. METHODS: Records of all patients with newly diagnosed Hodgkin lymphoma treated at our institution between 1991 and 2004 were reviewed. Pericardial involvement was identified by computerized tomography (CT) as focal thickening or nodularity present at the time of diagnosis, and by echocardiography as pericardial effusion. Outcomes measured were incidence of pericardial involvement, relapse-free survival, and overall survival. RESULTS: Thirteen of 273 patients (5%) had pericardial involvement. All patients with pericardial involvement had nodular sclerosing tumors versus 183 of 260 patients without pericardial involvement (P = 0.02); 9 (67%) had a bulky mediastinal mass versus 27% (P = 0.002). Two patients required pericardial drainage to drain very large effusions (n = 2). Both patients were symptomatic with either shortness of breath or superior vena cava syndrome. In the 11 cases that did not undergo surgical drainage, the effusion resolved within days after starting chemotherapy. Two patients experienced distant relapse but underwent successful salvage therapy. All 13 patients remain alive and free of disease at a median follow-up of 9.7 years (range, 1.7-12.9 years) with normal cardiac function. CONCLUSIONS: Pericardial involvement by lymphoma is usually asymptomatic unless accompanied by substantial pericardial effusion. In most cases, pericardial involvement resolves with treatment of the underlying malignancy, but close observation for hemodynamic complications is required. A symptomatic effusion, once treated, does not affect survival.