Primary epidural B-cell lymphoma (PEBL) is a rare neoplasm that presents initially with involvement of the epidural space. This study aimed to characterise the clinicopathological features of PEBL through a retrospective analysis of cases diagnosed at two institutions over a 15-year period. A total of 14 patients were identified, including seven women and seven men, with a median age of 63.5 years (range 21–76 years). These patients most often presented with symptoms of spinal cord compression, such as extremity paraesthesias, pain, and weakness. The thoracic region or thoracolumbar region was commonly involved in 10 (71%) patients. Eight (57%) patients had Lugano stage I disease, and 6six (43%) had stage II disease. Nine (64%) patients had a low or low-intermediate International Prognostic Index (IPI) score, and four (29%) patients had a high-intermediate or high score. All patients underwent surgical excision of the epidural mass, and histological analysis showed diffuse large B-cell lymphoma (DLBCL) in eight patients, follicular lymphoma in five patients, and high-grade B-cell lymphoma (HGBL) in one patient. Immunohistochemical analysis showed that all 14 cases were positive for pan B-cell markers and negative for CD3. Using the Hans algorithm, seven DLBCL cases had a germinal centre B-cell (GCB) immunophenotype and one case had a non-GCB immunophenotype. Fluorescence in situ hybridisation (FISH) analysis performed on eight cases showed one case of HGBL with MYC and BCL6 rearrangements, four cases of DLBCL with isolated BCL2 (n=2) or BCL6 (n=2) rearrangements, and one case of DLBCL with BCL2 and BCL6 rearrangements. All 14 patients were treated with excision, 12 of whom were also treated with chemotherapy; three of these patients also received radiation therapy. One patient was treated with an autologous stem cell transplant and subsequently CAR-T therapy. Clinical follow-up was available for all patients with a median of 39.5 months (range 1–123 months). At the last follow-up, 13 patients were alive and in complete remission and one patient with HGBL died 9 months after diagnosis. We conclude that PEBL predominantly arises in older adults and most often affects the thoracolumbar region. Patients usually have a low or low-intermediate IPI score. The most frequent type of lymphoma is DLBCL with a GCB immunophenotype. In this cohort, most patients received chemotherapy and had a favourable prognosis.
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