Adolph Louisa, Stoecklein Veit M, Passerini Verena, Heide Michael, Karschnia Philipp, Zausinger Stefan, von Baumgarten Louisa, von Bergwelt-Baildon Michael, Tonn Jörg Christian, Stoecklein Sophia, Thon Niklas, Schichor Christian, Rudelius Martina, Weigert Oliver
Department of Internal Medicine III, Laboratory for Experimental Leukemia and Lymphoma Research (ELLF), Ludwig-Maximilians University (LMU) Hospital, Max-Lebsche-Platz 30, Munich, Germany.
Department of Neurosurgery, Ludwig-Maximilians University (LMU) Hospital, Munich, Germany.
Ann Hematol. 2025 Sep 25. doi: 10.1007/s00277-025-06554-0.
Lymphomas confined to the spinal epidural space at initial diagnosis are rare and referred to as primary spinal epidural lymphoma (PSEL). This study presents clinical, histopathological, and molecular insights from 13 PSEL cases, including follicular lymphoma (PSEL-FL, N = 4), extranodal marginal zone lymphoma (PSEL-MZL, N = 3), diffuse large B-cell lymphoma (PSEL-DLBCL, N = 5), and Burkitt lymphoma (PSEL-BL, N = 1). Despite localized presentation at initial diagnosis, PSEL with aggressive histologies demonstrated remarkably unfavorable outcomes, contrasting the highly indolent clinical course observed in indolent PSEL, particularly PSEL-FL. Molecular analysis showed similarities to nodal counterparts and separation by entity. Comparison of PSEL-FL and classic FL revealed activated tumor microenvironment-associated pathways, potentially supporting extranodal lymphoma growth in this rarely affected spinal epidural site.
初诊时局限于脊髓硬膜外间隙的淋巴瘤很罕见，被称为原发性脊髓硬膜外淋巴瘤（PSEL）。本研究展示了13例PSEL病例的临床、组织病理学和分子学见解，包括滤泡性淋巴瘤（PSEL-FL，n = 4）、结外边缘区淋巴瘤（PSEL-MZL，n = 3）、弥漫性大B细胞淋巴瘤（PSEL-DLBCL，n = 5）和伯基特淋巴瘤（PSEL-BL，n = 1）。尽管初诊时表现为局限性，但具有侵袭性组织学特征的PSEL显示出非常不利的预后，这与惰性PSEL（特别是PSEL-FL）中观察到的高度惰性临床病程形成对比。分子分析显示与淋巴结对应物有相似性，并按实体进行区分。PSEL-FL与经典FL的比较揭示了与肿瘤微环境激活相关的途径，这可能支持在这个很少受影响的脊髓硬膜外部位发生结外淋巴瘤生长。
