RadioGraphics
Primary lymphomas of the central nervous system (CNS) represent one of the most enigmatic and deadly group of neoplasms. Their exact origin and classification are considerably debated among neuroscientists and provoke more questions than answers. What causes this tumor? Is it caused by a virus? Is it related to some other factor, possibly genetic? How can a tumor of transformed lymphocytes occur in an organ that contains no endogenous lymphoid tissue? How is it that this nodular-appearing tumor is in actuality diffusely infiltrative? Why is this tumor so exquisitely radiosensitive and steroid-sensitive at initial treatment that it completely disappears, as documented on follow-up radiologic images, only to aggressively reappear months later? Why has the biologic behavior of this tumor apparently changed since the early 1980s?
This article explores possible answers to these questions and presents the common radiologic features of primary intracranial CNS lymphoma with pathologic correlation based on the collection of cases contained in the Neuroradiology Archives of the Department of Radiologic Pathology at the Armed Forces Institute of Pathology. Historical observations of the disease and current treatment protocols and prognosis for patients are also discussed.