Primary CNS lymphoma represents about 1% of intracranial tumors. The incidence of primary

CNS lymphoma has been increasing steadily for the past two decades [16]. This increase is partially attributable to an increased prevalence of immunodeficient patients secondary to acquired immune deficiency syndrome (AIDS) and immunosuppressive treatment after organ transplantation. However, the incidence of the disease among immunocompetent persons has also increased. According to surveillance by the National Cancer Institute, the annual incidence of PCNSL increased from 2.7 cases/ten million persons in 1973-75 to 7.5 cases/ten million persons in 1982-84, even after exclusion of never married men as a relatively high-risk group for AIDS. Continuation of this trend produced an incidence of 30 cases/ten million persons in 1991-92 [17]. Although some of the increase may result from improved radiographic and immunologic detection, the trend antedates the widespread use of such technologies. Other etiologies for the observed increase in incidence of primary CNS lymphoma, such as environmental factors, remain speculative.

For immunocompetent patients, the sixth decade of life is the most common age of diagnosis of PCNSL [16,18]. The median age for presentation in the immunocompromised population falls within the fourth decade. Childhood disease is rare and is usually associated with congenital or acquired immunodeficiency.

In the immunocompetent population with primary CNS lymphoma, 60% of patients are male and 40% are female [16]. This is similar to the gender distribution of patients with systemic lymphoma. The immunocompromised


population with PCNSL is even more disproportionately male because of the male predominance in the AIDS epidemic.

Several risk factors for developing PCNSL have been identified (Table 16.5). Given its etiologic role in other lymphoproliferative disorders among immunosuppressed patients, the Epstein-Barr virus (EBV) may contribute to the development of primary CNS lymphoma in immunocompromised patients. EBV RNA has been found in CNS lymphoma tissue obtained from immunocompromised patients. Primary CNS lymphoma also occurs as a second malignancy after treatment of other malignancies, such as Hodgkin's lymphoma, non-Hodgkin's lymphoma and colon, breast and thyroid cancer. It is unclear whether this phenomenon reflects an underlying predisposition to cancer among these patients or it results from exposure to agents used to treat the initial malignancy.

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