Hodgkin Lymphoma Presenting With Spinal Cord Compression: Challenges for Diagnosis and Initial Management

Abstract

Hodgkin lymphoma (HL) can present with extra-nodal disease, but spinal cord compression is exceptionally rare. We describe a 15-year-old presenting with hip/back pain with normal initial examination. Persistent pain and raised inflammatory markers prompted further investigation with MRI, which revealed an epidural mass causing spinal cord compression. On examination, there was no palpable lymphadenopathy or cauda equina syndrome, but absent lower limb reflexes were noted. Following multidisciplinary discussion, it was determined that cauda equina syndrome was imminent and therefore surgical debulking was undertaken, both to prevent this complication and establish a diagnosis. At surgery, the tumor was highly vascular. Frozen section confirmed lesional material. Following surgery, and given the frozen section findings, a short course of steroids was commenced to reduce any peri-surgical edema. Unfortunately, histopathology was ultimately non-diagnostic, due to failure of immunohistochemistry on technically challenging material. Consequently, ultrasound-guided excision biopsy of a (non-palpable) cervical lymph node was performed five days later; histopathology showed typical effacement of the normal architecture and a conspicuous population of CD15/CD30-positive larger pale cells present, confirming nodular sclerosis classic HL, despite recent steroids. We review the available literature for HL presenting with spinal cord compression and describe the challenges for diagnosis and initial management in such cases.

Keywords: Hodgkin; cord compression; diagnosis; management; spinal cord.

Figure 1.

Representative MRI imaging of the epidural mass causing spinal cord compression. A, Sagittal T1 weighted contrast imaging demonstrating the anterior epidural enhancing mass in the lumbar canal (blue arrows) with enlarged paraaortic lymph nodes (white arrows). B, Axial T2 weighted imaging demonstrating enlarged paraaortic lymph nodes and abnormal paraspinal soft tissue (white arrows) and the anterior epidural mass (blue arrow). The thecal sac (red arrow) is displaced posteriorly and compressed.

Figure 2.

Representative pathologic findings in the spinal cord compression Hodgkin lymphoma case. Original (epidural) biopsy images showing (A) architecture (x40 magnification), (B) eosinophils and atypical cells (x400) and immunohistochemistry: (C) negative PAX-5 staining (x200) and (D) positive CD30 staining (x400). Images of the second (right cervical lymph node) biopsy revealing (E) atypical large cells and eosinophils (x400) and (F) large, atypical pale cells with PAX-5 staining (x400). Staging bone marrow images showing (G) replacement of the normal bone marrow with a malignant infiltrate (x40) and (H) eosinophils and atypical cells (x400). These atypical cells were positive for CD30 staining, consistent with stage 4 Hodgkin lymphoma.