CT of posttransplantation lymphoproliferative disorder in pediatric recipients of lung allograft

OBJECTIVE. The purpose of this study was to determine the CT and clinical findings of posttransplantation lymphoproliferative disorder in pediatric lung allograft recipients. MATERIALS AND METHODS. We reviewed the medical records and CT examinations of 260 lung transplantations in pediatric patients and found 26 recipients who had 29 episodes of histologically proven posttransplantation lymphoproliferative disorder. The clinical and CT features of the disease, the time to diagnosis, and the outcomes were assessed. RESULTS. The clinical presentation of posttransplantation lymphoproliferative disorder varied from asymptomatic pulmonary nodules (14/29 [48%]) detected on chest CT to specific (organ-related) and nonspecific symptoms (15/29 [52%]). Intrathoracic posttransplantation lymphoproliferative disorder occurred in 20 (69%) of 29 cases and manifested as multiple pulmonary nodules (n = 17), alveolar infiltrates (n = 2), and combined nodules and infiltrates (n = 1). In eight (28%) of 29 cases, there was extraparenchymal disease, including adenopathy, pleural effusion, and esophageal thickening and erosions. Extrathoracic posttransplantation lymphoproliferative disorder occurred in 13 cases and involved the abdomen (n = 10), paranasal sinuses (n = 2), and brain (n = 1). In the abdomen, extranodal disease was more common than nodal disease and presented as bowel wall thickening, focal mass lesions, and splenomegaly. In 18 of 29 episodes of posttransplantation lymphoproliferative disorder, the histologic diagnosis was lymphoma. The median time to diagnosis after transplantation for the 29 episodes of posttransplantation lymphoproliferative disorder was 10 months. Thirteen of the 26 patients died. The median time of survival after the diagnosis of posttransplantation lymphoproliferative disorder was 17 months. CONCLUSION. Posttransplantation lymphoproliferative disorder in pediatric lung transplant recipients occurs with relatively high frequency in both the chest and abdomen, tends to have lymphomatous features, and results in substantial mortality rates.