Extranodal marginal zone non-Hodgkin lymphoma mucosa-associated lymphoid tissue type with thoracic vertebral spine involvement: a sistemátic review of the literature and a case report from a university hospital in Brazil

Abstract

Background: Primary bone lymphomas are rare, accounting for less than 1% of non-Hodgkin's lymphomas (NHL). Extranodal marginal zone non-Hodgkin lymphoma of mucosa-associated lymphoid tissue (MALT) arising in the vertebral spine is extremely rare, with only seven cases reported. Methodology: A systematic literature review was conducted on cases from 2012 to 2022 of extranodal marginal zone MALT lymphoma involving the vertebral spine and associated with spinal cord compression. Additionally, a case report from a university hospital in Rio de Janeiro is described. The study was approved by the Research Ethics Committee (approval number: 5.818.416). Searches were performed in SciELO, PubMed, and LILACS databases, using the Health Sciences Descriptors: ("Vertebral Body" OR Spine) AND ("Lymphoma B-Cell Marginal Zone" OR "MALT Lymphoma" OR "Lymphoma of Mucosa Associated Lymphoid Tissue" OR "Mucosa Associated Lymphoid Tissue Lymphoma"). Articles with full texts available in English or Portuguese were selected. Case Description: A 56-year-old female presented with right-sided back pain, progressing to right flank irradiation, lower limb weakness (LLLL), and constitutional symptoms. MRI revealed a solid extradural mass occupying two-thirds of the vertebral canal's diameter, displacing and compressing the dorsal cord to the left. A bone marrow biopsy confirmed infiltration. Laminectomy was performed, and histopathology identified stage IVB extranodal marginal zone MALT lymphoma. Adjuvant chemotherapy led to complete remission. Results and Discussion: The literature review uncovered two relevant case reports. The thoracic spine was the most commonly affected region, and LLLL weakness was the most frequent symptom, with normal laboratory results. Treatment typically involved surgical tumor removal combined with radiotherapy or chemotherapy. Recurrence occurred in both cases, but follow-up showed no evidence of disease. The clinical presentation and exam findings of the current patient were similar to those in the literature, though with bone marrow infiltration. Treatment was consistent with cases in the review, with no recurrence to date. Conclusion: This case represents an uncommon manifestation of primary extranodal marginal zone MALT lymphoma with spinal cord compression, distinguished by bone marrow infiltration—a feature not previously reported in the two cases found in the literature. The case may be considered within the same group of cases reviewed.