A Conservative Approach to the Treatment of a Rare Case of Cervical Spine Double Expressor Diffuse Large B-cell Lymphoma: A Case Report

Abstract

Non-Hodgkin's lymphomas are a group of lymphoid neoplasms, with diffuse large B-cell lymphoma (DLBCL) being the most common subtype. Genetic alterations involving c-MYC, BCL-2, and BCL-6 have been implicated in the pathogenesis of subtypes of DLBCL with poor prognostic implications. This case report demonstrates a retropharyngeal mass with extension through the bilateral neuroforamina into the epidural space and posterior elements of the cervical spine (C2-C3), for which biopsy revealed diffuse large B-cell lymphoma. Here we present a unique case as it provides a solution for the dilemma on how to treat a patient with a known prior malignancy (gastrointestinal [GI] melanoma) with a retropharyngeal mass with epidural extension (dumbbell-shaped tumor) with an inconclusive initial CT-guided needle-core biopsy. A CT-guided biopsy only yielded that the mass was neoplasm; we had a choice between attempting gross total resection of the mass or open biopsy. Attempting gross total resection would have entailed an anterior approach (transoral with possible odontoidectomy or endoscopic endonasal with possible odontoidectomy) along with posterior instrumentation and fusion from occiput to C3, which is a rather morbid procedure that would subject the patient to a decreased quality of life as well as risks of vascular injury, dysphagia, and infection. We elected to perform an open biopsy of the epidural component of the mass through a decompressive laminectomy, which allowed for decompression of the spinal cord as well as a sampling of the mass. This provided treatment for possible increasing epidural compression from the mass, as well as diagnostic tissue. A multidisciplinary team discussed the case and developed a treatment plan for the patient with systemic and intrathecal chemotherapy in combination with radiotherapy.

Keywords: conservative approach; diffuse large b cell lymphoma; double expressor spinal dlbcl; spinal lymphoma; spinal tumours.

Figure 1. Magnetic resonance imaging (MRI) of the mass lesion

A: sagittal T2 showed a retropharyngeal mass at the level of C2 extending to the C3 level. B: axial T1 postcontrast showed moderate to severe central canal narrowing.

Figure 2. Needle core biopsy of the retropharyngeal mass lesion

A: microscopically, at low magnification, hematoxylin and eosin (H&E) stain showed highly cellular neoplasm. B: at higher power, H&E stain showed small to medium size cells in the fibrous background with large atypical epithelioid cells. C: CD45 was strongly positive in an obscuring background benign lymphocytes with some atypical cells negative.

Figure 3. Epidural mass lesion

A: microscopically, at low magnification (200x), the hematoxylin and eosin (H&E) stain showed a highly cellular lymphoid mass with small benign reactive appearing lymphocytes. B: at higher power (600x), small lymphocytes and large atypical cells with prominent nucleoli and large cytoplasm were seen.

Figure 4. Immunohistochemistry stain showed the immunopositivity of the atypical neoplastic cells for CD20 and PAX5.

The large atypical cells were also positive for CD20, PAX5, BCL-6, and c-MYC.