RESUMO
PURPOSE: Primary mediastinal B-cell lymphoma (PMBL) is recognized as a separate disease entity in the REAL classification. There is uncertainty about the relevance and especially the optimal management of this disorder. In this review, we discuss clinical presentation, pathology, pathobiology, and management of PMBL in order to provide a platform for initiation of further clinical and biologic studies. MATERIALS AND METHODS: Studies were identified through a MEDLINE search and from the bibliographies of relevant articles. RESULTS: PMBL is a tumor of young adults who present with a rapidly proliferating tumor. At diagnosis, the tumor is usually limited to intrathoracic organs. Spread to parenchymal organs such as liver, kidneys, and CNS is common at recurrence. Fibrosis and the presence of so-called clear cells are distinctive morphologic features of PMBL, although they are not pathognomonic or even necessary for diagnosis. On the other hand, biologic features relating to oncogene rearrangement and overexpression as well as cytogenetic features clearly differentiate PMBL from other types of diffuse large B-cell lymphoma. Phase II studies and retrospective series indicate cure rates that range from 38% to 88%. The role of chemotherapy, radiation, and high-dose chemotherapy with stem cell rescue in the management of PMBL is reviewed. CONCLUSION: PMBL has unique clinical and biologic characteristics that are radically different from those of other types of diffuse large B-cell lymphoma. Although relatively rare, its aggressive growth and its occurrence in young patients increase the clinical relevance of this entity. The consistent recognition and study of PMBL will allow the development of new approaches to its management.
