title: Immunoglobulin G4-related disease mimicking an epidural spinal cord tumor: case report.
abstract: The authors report a case of immunoglobulin G4-related disease (IgG4-RD) presenting as a paraspinal, epidural mass. This disease encompasses a host of autoimmune conditions that were previously thought to be separate entities. It is characterized by fibrosis, mediated by the aberrant proliferation and tissue invasion of IgG4-positive plasma cells, which can occur in any organ. As with other autoimmune conditions, it tends to be responsive to steroids and other immunosuppressants. It can rarely present as a tumefactive lesion of the central nervous system, creating the potential for misdiagnosis (given its similar radiological appearance to malignancy) and mistreatment. In 2015, a panel of experts convened to set forth guidelines for the diagnosis and treatment of IgG4-RD. In the case presented here, the patient initially presented with pain and weakness in the left upper extremity. Initial neuroimages revealed a contrast-enhancing mass extending from C-4 to T-1, invading the epidural spinal canal, encasing the exiting nerve roots, infiltrating the paraspinal musculature, and surrounding the left vertebral artery. A PET scan confirmed the mass was hypermetabolic, but results of fine-needle aspiration and CT-guided biopsy were inconclusive. Open biopsy yielded fibrotic tissue that met the pathological criteria for IgG4-RD: lymphoplasmacytic infiltrate, fibrosis in a storiform pattern, and obliterative phlebitis. The patient was treated with 2 doses of 4 mg of dexamethasone (Decadron) and then 50 mg of prednisone per day. Within 2 weeks, the mass was radiologically shown to have drastically decreased in size. The prednisone dose was decreased to 40 mg per day, and 100 mg of azathioprine per day was added. The patient continued to improve and the mass continued to decrease over the next 6 months. Currently, she has been weaned from all steroids and will be maintained on a daily dose of 100 mg of azathioprine.
authors: College of Medicine;
authors: Michelle M
authors: MM
authors: Williams
authors: Departments of 2 Neurological Surgery.|Department of Neurological Surgery, Ain Shams University, Cairo, Egypt.
authors: Hazem
authors: H
authors: Mashaly
authors: Neuro-oncology, and.
authors: Vinay K
authors: VK
authors: Puduvalli
authors: Pathology, The Ohio State University James Cancer Hospital and Wexner Medical Center, Columbus, Ohio; and.
authors: Ming
authors: M
authors: Jin
authors: Departments of 2 Neurological Surgery.
authors: Ehud
authors: E
authors: Mendel
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007074:Immunoglobulin G; D008875:Middle Aged; D019547:Neck Pain / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D013116:Spinal Cord / Q000000981:diagnostic imaging; D013120:Spinal Cord Neoplasms / Q000175:diagnosis*
chemical_list: D007074:Immunoglobulin G
keywords: ACDF = anterior cervical discectomy and fusion; CNS = central nervous system; IHC = immunohistochemical; IgG4-RD = immunoglobulin G4–related disease; IgG4-related disease; autoimmune disease; epidural mass; immunoglobulin G4; paraspinal mass; spinal cord compression