Rare-03primary dural lymphoma: single institution study of treatment and outcome

BACKGROUND: Primary dural lymphoma (PDL) is a rare form of primary intracranial lymphoma. Clinical and radiographic presentation often suggest a meningioma. Pathology often shows a low grade B-cell marginal zone lymphoma (MZL). MZL is very radiosensitive to relatively low dose of RT. Previous series have reported a high risk for systemic recurrence. METHODS: A retrospective review of primary dural lymphoma treated at the University of Miami Heath System between 08/2004-10/2014. RESULTS: Eleven patients were identified. Six were women. Median age at diagnosis was 48 years (range: 30 to 68). The most frequent symptoms at presentation were seizure (40%), headache (40%) and cranial nerve palsies (37%). Brain-MRI showed a single extra-axial enhancing lesion in 9 patients and multiple in two. Work-up was negative for systemic and leptomeningeal disease in all patients. Two patients had a history of HIV at diagnosis. All patients underwent resection/biopsy with an initial diagnosis of meningioma. Pathology showed marginal zone B-cell lymphoma (MZL) in all patients. One patient refused RT and was treated with rituximab. Ten patients were treated with focal RT. One of these patients refused to continue RT after been treated with 16 Gy. All others received between 30.6 to 36 Gy. Nine patients achieved complete response (CR) and two were lost to follow-up. Median follow-up was 3.3 years (range, 0.7 to 10 years). No patient developed a systemic relapse. Two patients developed CNS recurrences. One of these patients had progression of disease after being treated with 16 Gy and had a second course of RT (36 Gy), achieving CR. Another patient was treated with bendamustine/rituximab at progression and achieved CR. All patients are alive. CONCLUSIONS: Patients with PDL have a good prognosis. CR can be achieved with relatively low dose focal RT. No systemic relapses were observed in our series.