Sporadic Burkitt Lymphoma of the Thoracic and Lumbar Spinal Canal in an Adult: Oncogenicity and a Literature Review

Abstract

Burkitt lymphoma (BL) is an aggressive form of lymphoma that occurs due to translocation of the c-myc proto-oncogene on chromosome 8. BL is characterized by three distinct groups: African/endemic variant, immunosuppressive variant, or sporadic variant. Most cases of the sporadic variant occur in patients less than 40 years of age with a median age of 30 at diagnosis and are primarily seen in Caucasians. An immunocompetent 69-year-old male presented with subacute onset weakness in the lower extremities. Magnetic resonance imaging (MRI) of the lumbar spine revealed a mass in the right paraspinal musculature with epidural extension, neural foraminal narrowing, and severe spinal canal stenosis in L2-L5. MRI of the thoracic spine revealed significant T5-T6 cord compression due to metastatic masses. Further diagnostic imaging revealed diffuse lymphadenopathy within the mediastinum and abdomen. Subsequently, the patient underwent a core needle biopsy of the left axillary lymph node, which revealed cluster of differentiation 20 and 10 (CD20 and CD10), c-myc, and B-cell lymphoma 6 (Bcl-6) positive lymphoid cells. A diagnosis of BL was made. The patient was treated with oral steroids and received one round of radiation therapy. The patient opted to forgo any antitumor treatment and was discharged to hospice. Primary lymphomas of the central nervous system (CNS) account for <5% of all CNS tumors. Approximately 5-10% of CNS lymphomas are recorded as BL, with the majority classified as high-grade B-cell lymphomas. Paraspinal involvement with BL is rare and not commonly seen in the sporadic variant.

Keywords: burkitt lymphoma; codox; epidural; ivac; neurology; oncology; radiology; spinal canal; spinal cord; sporadic burkitt lymphoma.

Figure 1. MRI of the thoracic and lumbar spine.

(A) MRI T2 sagittal view of the lumbar spine revealing L2-L5 spinal canal stenosis. (B) MRI T2 sagittal view of the thoracic spine revealing significant cord compression at T5-T6 due to ventral and dorsal epidural masses. (C) MRI T2 coronal view of the thoracic spine revealing heterogeneous masses measuring 9.2 × 2.3 cm likely representing metastatic disease. (D) MRI T2 axial view of the lumbar spine revealing a mass measuring approximately 3.1 × 4.1 cm in the right paraspinal musculature with direct epidural extension and neural foraminal narrowing. MRI: magnetic resonance imaging

Figure 2. CT scan of the chest, abdomen, and pelvis.

(A) Bilateral axillary lymphadenopathy. (B) A 1.0 cm rounded soft tissue nodule along the fascial plane between the right gluteus medius and maximus muscles is significant for metastatic disease. CT: computerized tomography

Figure 3. Histology and immunohistochemistry of the left axillary lymph node biopsy.

(A) Medium-sized lymphoid cells with many mitotic figures. There are numerous tangible body macrophages admixed within the lymphoid cells. (B) High-power image of the previous slide. (C) Immunohistochemical stain is positive for cluster of differentiation 10 in blue cells. (D) Immunohistochemical stain is positive for B-cell lymphoma 6 in blue cells. (E) Immunohistochemical stain is positive for c-myc in blue cells.

Figure 4. Reciprocal translocation t(8;14)(q24;q32) leading to the activation of the c-myc protoncogene.

MYC: Myc Proto-oncogene. IG: immunoglobulin

Figure 5. Oncogenic mechanisms associated with the EBV.

EBV: Epstein-Barr virus; EBNA-1: Epstein-Barr virus nuclear antigen 1; EBNA-2: Epstein-Barr virus nuclear antigen 2; LMP-1: latent membrane protein 1; LMP-2: latent membrane protein 2; NOX-2: reduced nicotinamide adenine dinucleotide phosphate oxidase 2; RAG-1/2: recombination activating gene 1/2; IG: immunoglobulin; c-MYC: cellular-Myc proto-oncogene