Primary mediastinal large cell lymphoma in children: a report from the Childrens Cancer Group

Malignant lymphomas arising in the mediastinum account for approximately 60% of all mediastinal tumors in children; two-thirds are non-Hodgkin's lymphomas and one-third represent Hodgkin's disease. In contrast to adults, in children mediastinal non-Hodgkin's lymphomas are usually synonymous with lymphoblastic lymphoma, and nonlymphoblastic lymphomas are rare. We describe nine children with primary mediastinal large cell lymphoma who were treated with the Children's Cancer Group protocol CCG-503, a randomized phase III protocol for disseminated nonlymphoblastic lymphoma. Histologic subclassification revealed three immunoblastic lymphomas, three multilobated large cell lymphomas, one with clear cell features, and two large noncleaved cell lymphomas. Sclerosis, of variable degrees, was seen in all tumors. Immunophenotyping revealed all tumors to be of B cell lineage. Thymic epithelial cells could be demonstrated, utilizing antibody to keratin, in two of nine patients, suggesting that some of the tumors are of thymic origin. None of the patients had central nervous system or bone marrow involvement. It appears that primary mediastinal nonlymphoblastic lymphomas in children, although much less common, are similar to those seen in adults. These tumors must be differentiated from lymphoblastic lymphoma and Hodgkin's disease, as the therapeutic approach may depend on histologic subtype. Primary mediastinal large cell lymphoma in children appears curable with aggressive treatment in the majority of patients.