Primary Spinal Marginal Zone Lymphoma - Case Report and Review of the Literature.

Feb 2012

Ahmadi SA, Frank S, Hänggi D, Eicker SO.

Source

1Department of Neurosurgery, Heinrich-Heine-University, Düsseldorf, Germany, Email: ahmadi@med.uni-duesseldorf.de, Phone: +49 176 66 88 9045, Fax: +49 211 81 16509 2Division Neuropathology - Institute of Pathology, University Hospital Basel, Schönbeinstr. 40, CH-4031 Basel 3Department of Neurosurgery, Heinrich-Heine-University, Düsseldorf, Germany. Email: sven.eicker@med.uni-duesseldorf.de.

Abstract

BACKGROUND AND IMPORTANCE:

Marginal zone lymphoma (MZL) describes a heterogeneous group of indolent B-celllymphomas. The World Health Organization recognizes 3 types of MZLs: splenic MZL (SMZL), nodal MZL (NMZL) and extranodal MZL of mucosa-associated lymphoid tissue (MALT). 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 male suffering from 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 ofmarginal zone B-cell lymphoma (MZBCL), adjuvant radiotherapy was employed, with some combining radiotherapy and chemotherapy after surgical removal of the main tumor bulk. 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 employed in larger case series. While there is no defined treatment guideline for this rare disease entity, our review of the literature suggests a favorable prognosis when combining both surgical and adjuvant radiotherapy approaches.

Neurogsurgery