Objective: A description of clinical features and treatment results in patients with a central nervous system (CNS) localization of systemic non-Hodgkin's lymphoma (NHL).
Design: Retrospective.
Setting: Department of Neuro-oncology and Haematology, Daniel den Hoed Cancer Centre, Rotterdam, the Netherlands.
Methods: All patients with NHL (but not primary CNS lymphoma) treated in the period January 1st, 1990-December 31st, 1996 at the department of neuro-oncology were reviewed for presence of CNS localizations. Clinical characteristics like malignancy grade, disease stage, presence of extranodal localizations, B-symptoms, serum LDH, neurological signs and symptoms, results of ancillary investigations, treatment and response were recorded.
Results: In this period 25 patients were diagnosed with leptomeningeal lymphoma, 2 with an intracerebral lymphoma, and 7 with both. In almost all patients the CNS localization developed during systemic progression of the NHL. Most patients presented with a radicular syndrome, cranial nerve deficits, headache or encephalopathy. More than 80% of the patients showed clinical improvement after treatment with intrathecal chemotherapy, radiation therapy or a combination of both. The median survival was three and a half months, six month survival was 32%. Progression of systemic disease was the most frequent cause of death.
Conclusion: In most patients good palliation of neurological signs and symptoms could be obtained with intrathecal chemotherapy and radiation therapy. Survival in these patients was limited, however, in part because of the frequent concurrent progression of systemic disease. Patients with CNS localizations with chemotherapeutic treatment possibilities with a realistic chance of cure or longterm survival should be treated accordingly. Otherwise, treatment should consist of intrathecal chemotherapy or radiation therapy of involved areas only.