Primary spinal marginal zone lymphoma

BACKGROUND AND IMPORTANCE: Marginal zone lymphoma (MZL) describes a heterogeneous group of indolent B-cell lymphomas. The World Health Organization recognizes 3 types of MZLs: splenic MZL, nodal MZL, and extranodal MZL of mucosa-associated lymphoid tissue. There is no consensus on the optimal adjuvant treatment modalities for intracranial primary MZLs. To date, no case of spinal primary MZL has been reported.

CLINICAL PRESENTATION: We present the first case of spinal MZL diagnosed in a 65-year-old man with progressive paraparesis. He underwent surgical removal of the main spinal tumor mass, which extended epidurally from vertebral body T3 to T7. Surgery was followed by 10 sessions of local irradiation for a total dose of 31 Gy. On long-term follow-up in 2010, the patient was in good health without any signs of residual or recurrent disease. Twenty-seven publications reporting on 61 cases of intracranial primary MZL were identified and reviewed. In the majority of cases of marginal zone B-cell lymphoma, adjuvant radiotherapy was used, with some combining radiotherapy and chemotherapy after surgical removal of the bulk of the main tumor. Long-term follow-up in most patients showed no evidence of disease and clinical well-being years after the initial diagnosis.

CONCLUSION: Chemotherapy and/or radiation have been used in larger case series. Although there is no defined treatment guideline for this rare disease entity, our review of the literature suggests a favorable prognosis when combining surgical and adjuvant radiotherapy approaches.