Background: The high incidence of local failure and the lack of evidence of systemic dissemination of primary central nervous system lymphoma (PCNSL) has led some authors to conclude that complete staging with extensive tests is not necessary in patients with a lymphomatous cerebral mass who present with a focal neural syndrome. Moreover, De Angelis emphasizes that there is no report in the literature of systemic lymphoma presenting as a cerebral mass lesion, whereas others conclude that an extensive systemic evaluation is unnecessary or that the staging should be limited to physical examination, routine blood studies, and a chest radiography.
Methods: In 16 PCNSL patients observed between 1982 and 1992, we found two patients showing systemic involvement of lymphoma after staging workup with chest X-ray, total body computerized tomographic (CT) scan, cerebrospinal fluid cytology examination, and bone marrow biopsy. In one patient, a CT abdominal scan showed retroperitoneal and pelvic lymphonodal involvement, whereas in the second patient, bone marrow biopsy was positive. These systemic onsets were found no later than 2 months after the diagnosis of central nervous system involvement, which is sufficient time to conclude that these locations were present at the diagnosis of the cerebral mass.
Results: In the present study, complete staging demonstrated widespread dissemination (lymph nodes or bone marrow) in two patients with initial neurologic symptoms and a cerebral mass lesion.
Conclusions: In our experience, complete staging allowed detection of systemic lymphoma in two patients initially considered to have PCNSL.