Primary central nervous system lymphoma (PCL) is an uncommon variant of extranodal non-Hodgkin lymphoma (NHL) that involves the brain, leptomeninges, eyes, or spinal cord without evidence of systemic disease.
The treatment, complications, and prognosis of PCL will be reviewed here. The clinical and pathologic features of this disorder, as well as the treatment of secondary involvement of the CNS by lymphoma originating outside the CNS, are discussed separately. In addition, a discussion of PCL in HIV positive patients is presented separately. (See "Clinical presentation, pathologic features, and diagnosis of primary central nervous system lymphoma" and "Clinical presentation and diagnosis of secondary central nervous system lymphoma" and "AIDS-related lymphomas: Primary central nervous system lymphoma".)
The pretreatment evaluation of patients with PCL both determines the extent of the disease and provides information about the individual's comorbidities that are likely to have an impact on treatment options. In addition to a history and physical examination, it is our practice to perform the following pretreatment studies in patients with PCL:
●Laboratory studies including a complete blood count with differential, chemistries with liver and renal function and electrolytes, lactate dehydrogenase (LDH), and serologic testing for HIV.
●Full ophthalmological evaluation including slit lamp examination of both eyes is strongly recommended, even in the absence of visual symptoms, since ocular lymphoma occurs in up to 20 percent of patients, and may be followed by reseeding of brain sites. (See "Clinical presentation, pathologic features, and diagnosis of primary central nervous system lymphoma", section on 'Slit lamp examination'.)