Objective: Mucosa-associated lymphoid tissue (MALT) lymphoma, also known as extranodal marginal zone lymphoma (MZL), is a subtype of indolent B-cell non-Hodgkin's lymphoma (NHL). MALT lymphomas are encountered mainly in mucosal organs such as the stomach, however, they can also be found in non-mucosal organs and tissue regions. MALT lymphoma of the spinal dura is a very rare condition. Here, we present the clinical presentation pattern, histopathologic and radiographic findings, treatment options, and response to treatment in a rare case of isolated primary spinal MALT lymphoma.
Case report: A 74-year-old male presented to our hospital with progressive weakness and loss of sensation in bilateral lower extremities, and fecal and urinary incontinence. Spinal MRI examination visualized an extra-axial mass lesion of approximately 45mm×11mm between the vertebral levels T5 and T7. The lesion markedly compressed the spinal cord, severely narrowing the spinal canal and bilateral neural foramina. In order to ensure early decompression of the spine and histopathological diagnosis of the epidural mass, a total laminectomy of T6 and a subtotal resection of the mass were performed. On immunohistochemical examination of the mass, neoplastic cells showed: LCA(+), CD20(+), CD79a(+), PAX5(+), bcl-6(−), fascin(−), CD3(−), CD5(−), cyclin D1(−),CD23(−), CD138(−), kappa (−), lambda (−), MUM1(−), CD10(−), tdt(−), CD15(−), CD30(−), reticulin(−), and a Ki67 proliferation index of 20%; and the pathology department reported the findings to be consistent with MALT lymphoma of the dura. Following mass resection, FDG-PET CT) was performed to determine the extent of the disease, and other regions of the body did not show 18-FDG uptake. Bone marrow aspiration and biopsy showed that there was no infiltration. Only systemic chemotherapy was planned as the patient refused to undergo radiotherapy. A systemic combination therapy with R-CHOP protocol every 3 weeks and central nervous system prophylaxis with intrathecal cytarabine and dexamethasone were carried for the patient. After two chemotherapy cycles, there was a significant improvement in motor weakness and the fecal and urinary function impairment. After a total of 6 cycles, spinal MRI and FDG-PET CT showed complete disappearance of the lesion. The patient remains in remission, at 1-year follow-up.
Conclusion: This report presents a case of primary spinal MALT lymphoma, which is extremely rare. Lymphoma should be considered in the differential diagnosis of patients who present with a spinal mass and the subtype of the lymphoma must be identified. The management of MALT lymphomas is quite heterogenous and there exist no universally-accepted therapeutic guidelines for this rare condition. A treatment option must be selected in consideration of the disease subtype, stage, and the clinical characteristics of the patient. In spinal MALT lymphoma, both local and systemic treatment options are available. Local treatments such as surgical resection or radiotherapy can achieve complete remission in patients with MALT lymphomas confined to a single site or at early stages. Systemic treatment is an option for patients who are not suitable for local treatment and appropriate patients may be administered systemic chemotherapy regimens that include anti-CD20 monoclonal antibodies.
