Primary spinal mucosa-associated lymphoid tissue lymphoma

RATIONALE Mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent B-cell lymphoma which occurs mainly in the organs having mucosal layer and owns a fairly good prognosis. To date, 7 cases of spinal primary MALT has been reported before. However, there is no consensus on the optimal adjuvant treatment modalities for primary spinal MALT. The aim of this study was to add a new case of MALT which responded well to systemic therapy to the literature and to review the current literature. PATIENT CONCERNS A 68-year-old woman visited to our hospital due to back pain and progressive bilateral lower extremity weakness for 2 months. Magnetic resonance imaging (MRI) of the spine revealed a diffusely contrast-enhancing epidural mass extending from vertebral body T6 to T8 with compression of the spinal cord. Due to the spinal cord compression, patient underwent surgical resection. Histological examination indicated monocytoid small B-cells. Immunochemical study demonstrates that most tumor cells were positive for CD20, CD21, CD45, CD79a, CD43, bcl-2 with Ki-67 labing index was 15%, but were negative for CD3, CD5 cyclin D1, BCL6, and CD23. The positron emission tomography/computer tomography (PET/CT) revealed that right iliac wing and right liver were metastases for the standard uptake value (SUV) were 9.05 and 8.35, respectively. DIAGNOSES Based on these findings, final diagnosis of spinal MALT lymphoma was made. INTERVENTIONS After the diagnosis, the patient received 6 cycles of immuno-chemotherapy and repeated intrathecal methotrexate and intrathecal cytarabine. OUTCOMES At 1 year follow up, no recurrence or other dissemination was detected. LESSONS Chemotherapy and/or radiation have been employed in larger case series. While there is no defined treatment guideline for this rare disease entity, our reported case suggests a favorable prognosis when combining both surgical and adjuvant systemic approach.