Subaxial cervical Castleman’s disease: A rare cause of myelopathy

Abstract

Background: Castleman’s disease (CD) is a rare lymphoproliferative disease of unknown origin which rarely affects the spine. Here, we present CD involving a lytic, destructive C3 lesion with extension into the spinal canal contributing to upper cervical cord compression. Notably, the lesion mimicked other primary bone lesions, metastatic tumors, and/or lymphoma. Case Description: A 52-year-old male presented with progressive quadriparesis (i.e. weakness, instability of gait) and loss of dexterity in both hands over 2 weeks. The MRI, X-ray, and CT scans revealed a destructive lytic lesion involving the C3 vertebral body (i.e. including both anterior and posterior elements). The patient underwent a C3 total and C4 partial laminectomy followed by a C2-C4/5 instrumented fusion (i.e. included C2 pedicle screws/laminar screws, and C4/C5 lateral mass fixation). Histopathology showed a lymphoproliferative disorder with follicles of different sizes, central abnormal germinal structures, and a Mantle zone (i.e. expanded germinal centre with concentric layering with an “onionskin” appearance). These findings were all consistent with the diagnosis of CD (i.e. hyaline-vascular type). Conclusion: CD, a rare lymphoproliferative disease of unknown origin rarely affects the spine. Here, we presented a 52-year-old male with a C3 lytic lesion resulting in C3/4 cord compression that favorably responded to a C3/4 laminectomy with posterior instrumented fusion.