Clinical and molecular features of primary spinal epidural lymphomas.

IF 2.4 3区医学 Q2 HEMATOLOGY

Annals of Hematology Pub Date : 2025-09-25 DOI:10.1007/s00277-025-06554-0

Louisa Adolph, Veit M Stoecklein, Verena Passerini, Michael Heide, Philipp Karschnia, Stefan Zausinger, Louisa von Baumgarten, Michael von Bergwelt-Baildon, Jörg Christian Tonn, Sophia Stoecklein, Niklas Thon, Christian Schichor, Martina Rudelius, Oliver Weigert

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Abstract

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.

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原发性脊髓硬膜外淋巴瘤的临床及分子特征。

最初诊断局限于脊髓硬膜外腔的淋巴瘤是罕见的，被称为原发性脊髓硬膜外淋巴瘤（PSEL）。本研究报告了13例PSEL的临床、组织病理学和分子分析，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）。尽管在初始诊断时表现为局部症状，但具有侵袭性组织学的PSEL表现出非常不利的结果，与惰性PSEL（尤其是PSEL- fl）的高度惰性临床病程形成鲜明对比。分子分析结果表明，它们具有相似的节点对应物和实体分离。PSEL-FL和经典FL的比较揭示了激活的肿瘤微环境相关通路，可能支持结外淋巴瘤在这个很少受影响的脊髓硬膜外部位的生长。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Annals of Hematology 医学-血液学

CiteScore

5.60

自引率

2.90%

发文量

304

审稿时长

2 months

期刊介绍： Annals of Hematology covers the whole spectrum of clinical and experimental hematology, hemostaseology, blood transfusion, and related aspects of medical oncology, including diagnosis and treatment of leukemias, lymphatic neoplasias and solid tumors, and transplantation of hematopoietic stem cells. Coverage includes general aspects of oncology, molecular biology and immunology as pertinent to problems of human blood disease. The journal is associated with the German Society for Hematology and Medical Oncology, and the Austrian Society for Hematology and Oncology.