Primary Extradural Lymphoma with Marginal Zone Transformation- A Rare Presentation

Abstract

Background: Primary spinal extradural non-Hodgkin lymphoma is rare, and marginal zone transformation is extremely uncommon. We report a rare case of primary spinal EMZL with marginal zone transformation, without systemic or CNS involvement.

Case Presentation: We report a 38-year-old premenopausal female with type 1 diabetes who presented with 2 months of paraparesis and sensory loss below the umbilicus. MRI revealed patchy meningeal thickening from D1–L3, and PET-CT confirmed a localized thoracolumbar extradural lesion. Surgical D3–D6 laminoplasty with excision was performed. Histopathology and immunophenotyping confirmed follicular centre cell NHL with marginal zone transformation, with CD20, CD10, PAX5, CD23, BCL6 positivity and Ki-67 20% overall with a focal hotspot of 75%, indicating high-grade transformation. Differential diagnoses including mantle cell lymphoma, CLL/SLL, and pure follicular lymphoma were excluded. The patient received six cycles of modified R-GMALL chemotherapy with triple intrathecal therapy. Treatment was well tolerated, with only grade 2 neutropenia and grade 1 mucositis, and she achieved near-complete neurological recovery. Follow-up at 14 months demonstrated sustained neurological function and complete metabolic remission.

Conclusion: This case highlights the importance of early recognition, intensive chemoimmunotherapy, and careful supportive care in rare transformed MZL, supported by emerging prognostic models incorporating systemic inflammation markers.