Objective: Extranodal lymphomas, by definition, can involve any organ or tissue. Brain parenchyma, spinal cord, eyes, cranial nerves, and meninx are extranodal regions that show involvement at much lower rates. It is quite rare for lymphoma patients to present to the hospital with symptoms and findings associated with spinal cord compression as the initial presentation. This condition can lead to irreversible autonomic dysfunction, and motor and sensory loss. Here, we present a rare primary spinal intradural extramedullary diffuse large B-cell lymphoma (DLBCL) case who presented with acute neurological symptoms and no findings of cerebral involvement or involvement at any other site.
Case report: A 41-year-old male patient presented to our hospital with thoracic back pain and progressive complaints of weakness, numbness and difficulty in ambulation in bilateral lower extremities. On spinal MRI examination, a well-circumscribed intradural extramedullary mass with a craniocaudal extension of 6cm and an AP diameter of 1cm that was isointense to the spinal cord on T1-weighted sequences and slightly hyperintense on T2-weighted series, and showed diffuse homogenous contrast enhancement after intravenous contrast agent injection was determined between the vertebral levels T6 and T8. In the surgical operation, the mass showed partial invasion of the vertebral bone and the surrounding muscle. The mass invading the dura was resected and laminectomy was performed at T6-T9. On histopathological examination of the mass, there was diffuse malignant infiltration by large atypical lymphoid cells with prominent nucleoli and a coarse chromatin structure. On immunohistochemical examination, neoplastic cells showed; CD20 (+, diffuse), CD3 (−), MPO (−), Tdt (−), CD1a (−), S100 (−), ALK (−), CD68 (−), CK (−), actin (−), vimentin (−) staining, and the Ki67 proliferation index was 70%. The pathology department reported the mass to be consistent with a diffuse large B cell lymphoma (centroblastic type). Cervical-thoracic-abdominopelvic CT was performed to determine the extent of the disease, and no masses, organomegaly, or enlarged lymph nodes were detected. Bone marrow aspiration and biopsy did not show bone marrow involvement. The patient received chemotherapy consisted of R-CHOP and was administered with six cycles. After chemotherapy, radiotherapy was given at a total dose of 40Gy as 2Gy per fraction. The strength of the bilateral lower extremity muscle groups showed daily improvement and the patient was able to walk normally with two courses of chemotherapy, after approximately six weeks. The patient remains in remission without clinical or radiological relapse under follow up after nearly 3 years.
Conclusion: The differential diagnosis of patients who present with a spinal mass should be made carefully. It must be considered that, although rarely, DLBCLs can present as massive disease-causing spinal compression, and that clinically significant improvement can be achieved by timely and effective treatment. In patients who present with spinal compression, early decompression, particularly by means of surgery, is of great importance. Considering that spinal DLBCL is a malignant disease, appropriate treatment approaches play a vital role in achieving neurological recovery, longer survival times, and better life quality.
